33_organelles.txt

Limited proteolysis, phosphorylation, and isoprenylation all occur here. In flagellate protozoa, it is known as theparabasal body and in plants, it is known as the dictyosome. It was first described in 1898 but, because of thelimitations of light microscopy and because staining techniques failed to resolve its structure, its existence was notproven until the late 1950s. FTP, name the organelle where glycosylation occurs and proteins are packaged forsecretion.
Answer: Golgi apparatus or Golgi bodies or Golgi sacs Belgian biochemist Christian de Duve identified them in 1955 with the aid of an electron microscope. Originating asbuds on the trans-Golgi network, they have an acidic interior which can destroy other worn out organelles and cellularinvaders. For 10 points, name these organelles which take in extracellular molecules by fusing with endosomes.
Answer: lysosomes The outer membrane is dotted with the protein porin, while ubiquinone and cytochrome oxidase complex line thefolded inner membrane. The double membrane is explained by the endosymbiont hypothesis which also helps explainthe presence of self-replicating DNA, similar to that found in bacteria. For ten points, name the cellular organelleresponsible for aerobic respiration.
Answer: mitochondrion or mitochondria Composed of a stack of five to eight disk-shaped membraned vesicles, it is structurally polarized with a 'cis' face nearthe transitional region of the RER and a 'trans' face near the plasma membrane. The principal director of cellularprotein traffic is, for ten points, what organelle named for its 1898 discoverer, an Italian cytologist.
Answer: Golgi apparatus or complex or body They often stand out in electron micrographs because of a crystalloid core made up of urate oxidase. In germinatingseeds, they are called glyoxysomes, since they convert fats to sugars as part of the glyoxylate cycle. Remarkablyadaptable, they are small in yeast cells grown on sugar, but grow large if the yeast is transferred to methanol. For 10points-name these organelles that break down many molecules by producing hydrogen peroxide.
Answer: peroxisomes Swelling and the appearance of calcium deposits in this organelle signify that the cell is undergoing necrosis. Boundedby a smooth outer membrane, its inner membrane consists of several shelf-like folds called cristae. Possessing its owntype of DNA, it contains the machinery for synthesizing proteins. FTP, what is this organelle which provides energy foranimal cells.
Answer: mitochondrion (or mitochondria) Since these organelles differ in size and chemical properties between prokaryotes and eukaryotes, anitbiotics such astetracycline, streptomycin, and Zithromax can be used to target them and kill prokaryotic cells without hurtingeukaryotic cells. Each is composed of two subunits with characteristic shape, and contains a total of four RNAmolecules and about 70 different proteins. Manufactured in the nucleus and then released into the cytoplasm in orderto function, name these organelles that are directly involved in protein synthesis and which, FTP, are found floating inthe cytoplasm or attached to the endoplasmic reticulum.
Answer: ribosomes In 2002, this compound was the first to be used successfully to prevent muscle loss in certain lung cancer patients. Itcan be used to treat asthma and diabetes, and in the body it acts as a neurotransmitter, but its most important functionwas discovered by Fritz Lipmann and Herman Kalkar in 1941. In cells, it readily loses a phosphate ion by hyrdolysis,providing the means for organelles to carry out biological processes. The predominant source of energy in living cells,FTP what is this compound named in part for the D-ribose component's possession of three phosphate groups.
Answer: ATP or adenosine triphosphate A drug known as brefeldin A blocks the transfer of proteins to this organelle, while vesicles bound for it tend to becoated with either COP I or COP II. The current paradigm for its sorting abilities is explained by the "maturationalmodel", which states that proteins from the endoplasmic reticulum enter its cis face and are packaged in its cisternae,after which they are transported out of the trans face. Composed of a stack of flattened sacks, FTP, identify this cellularorganelle, an apparatus named after an Italian anatomist.
Answer: Golgi complex or apparatus Diseases caused by defects in these include hyperoxaluria, Refsum's disease, and Zellweger syndrome. Althoughinvolved in bile acid synthesis, amino acid metabolism and cholesterol synthesis, the most important function of theseorganelles may be the beta-oxidation of long chain fatty acids. FTP, name this type of organelle which contains enzymessuch as catalase and is named due to its role in the production and decomposition of hydrogen peroxide.
Answer: peroxisome(s) It is surrounded by a single membrane called the tonoplast. Its storage variety is generally responsible for holdingphenolics, acids, nitrogenous wastes, and anthocyanins away from the cytosol. Its contractile variety is used to pumpwater out of the cell quickly and plays a role in the cell's turgor pressure. Its food variety is responsible forphagocytosis. Generally smaller in animal cells, plant cells usually contain one large version of this organelle. FTP,identify this organelle that serves as a general repository for materials in cells.
Answer: vacuole Discovered in 1950 by Rene de Duve, mannose-6-phosphate tags molecules destined for it. It secretes a hydrogen ionATPase in order to acidify its immediate environment. Born from endosomes in receptor-mediated endocytosis, Pompeand Gaucher diseases are caused by its malfunction and a deficiency of hexosaminidase-A produced by this organellecauses Tay-Sachs disease. Involved in apoptosis, its components are produced by the rough ER and packaged by theGolgi body. FTP, name this organelle that digests macromolecules and releases lytic enzymes upon a cell's death.
Answer: lysosome These organelles are particularly abundant in renal proximal convoluted tubule cells and liver parenchyma cells. Boundby a single membrane, they contain type II oxidases like urate oxidase, whose action produces the compound for whichit is named. A predominant constituent is the enzyme catalase, which helps in the process of detoxification for whichthis organelle is known. FTP, what is this organelle named for the presence of the compound H2O2?Answer: peroxisomes The dense fibrillar component of this organelle is thought to be the location of synthesis of rDNA, which later expandsto form this structure. Transcriptional silencing of this rDNA leads to the production of pre-ribosomal RNAs, which arefurther modified by snRNAs to form mature ribosomal RNAs. These ribosomal RNAs are exported by the Cajal bodies tothe cytoplasm to form mature ribosomes. FTP, name this site of synthesis for ribosomes, located inside the nucleus.
Answer: nucleolus Cytotoxic T cells secrete of perforin from these structures and melanocytes secrete melanin from a modified form ofthem. In addition, their exocytosis provides the additional membrane needed to quickly seal wounds in the plasmamembrane. I-cell disease is caused by a failure to phosphorylate all enzymes that are supposed to be transported tothem. FTP, name these organelles that contain lipases, nucleases, proteases, and other digestive enzymes that breakdown complex molecules in the body.
Answer: lysosomes Lowe syndrome causes a deficiency of an enzyme which affects the metabolic processes that take place in this cellstructure. One of its main functions is the remodeling of oligosaccharides, while other functions include the synthesis of lipids like sphingomyelin and the addition of sulfate groups to tyrosine. Often located near endoplasmic reticulum exitsites, it uses clathrin-coated vesicles to send certain proteins to lysosomes and other vesicles to secrete proteins fromthe cell. FTP, name this organelle divided into stacks of flattened, membrane-bounded cisternae, which packages andsorts newly-created proteins.
Answer: Golgi Apparatus/Body/Whatever Since their DNA copying does not have any error checking, the probability of random mutation is much higher, leadingto diseases such as Kearn-Sayre syndrome. Its DNA typically consists of five to ten copies, all inherited from the mother,and when they divide these copies are randomly distributed between the offspring. Some theories hold that its DNA isderived from a single source called "Eve". FTP, what are these organelles long ago incorporated into eukaryotic cells toproduce energy for them?Answer: mitochondria In one of the forms of this, 6-Formylpterin enhances it by activating protein kinase C-prime, while it may be blocked byupregulation of HSP-70. It is initiated by the migration by Bak or Bax to Bcl-2 binding sites, or via binding to CD95 by Fasligand. Exposure of peroxidized phosphatidylserine occurs in its final stages, after caspase 9 binds to cytochrome c fromthe mitochondrion and a certain protease activates factor-1, leading to degradation of chromosomal DNA andbreakdown of cellular organelles into vesicles. FTP, name this process, which occurs in endometrial shedding duringmenstruation and finger formation, and is a form of programmed cell death.
Answer: apoptosis [a-po-TO-sis] (prompt on programmed cell death) Proteins destined for these structures are post-translationally modified to include mannose-6-phosphate residues ontheir N-linked oligosaccharides. The addition of the M6P tag to enzymes, such as aryl-sulfytase A, is being investigatedas a potential treatment for diseases that affect these organelles, which diseases include metachromaticleukodystrophy and Fabry's disease. Once inside these structures, the proteins and their M6P tags dissociate due to theenvironment created by vacuolar ATPase, which is closely related to the proton pump used in oxidativephosphorylation. FTP, identify these organelles containing hydrolases that function best at a pH of about 5 and whichare structurally similar to peroxisomes.
Answer: lysosomes Defects in these objects can cause Kearn-Sayre Syndrome or Luft Disease. They contain porins to allow for passivediffusion of selected molecules through their outer membranes. They provide evidence for Margulis' endosymbiotictheory and are important to evolutionary and genetic studies due to their derivation solely from the mother. They allowsome ribosomes in their matrix to allow for processes such as chemiosmosis and the citric acid cycle. FTP, name theseorganelles whose primary function is to convert organic materials into ATP, the so-called "powerhouses" of the cell.
Answer: mitochondria The first atomic structure of one of these was published by Ban et al. in 2000. Its leading end is labeled P and is followedby A. In prokaryotes, it is made of three strands and consists of 30S and 50S subunits; in eukaryotes it has four strands.
It uses an initiator called fMET and then works by using peptidyl transferase to bind two amino acids, creating a peptidebond; it does this by reading mRNA and using tRNA to create its product. FTP, these, sometimes located within themitochondria or in the cytoplasm, are, what protein-assembling organelles, most commonly located on the roughendoplasmic reticulum?Answer: ribosomes (accept 50S ribosomal subunits or large ribosomal subunits until "strands") Kearns-Sayre syndrome is caused by faults in this, and its normal direction of heritance, which is caused byubiquitination of it, can be reversed in certain species of Musa. Analysis of this commonly consists of sequencing of HVRregions and was used to establish woolly mammoth descent as described in a December, 2005 Nature article. Itsexistence strongly supports the endosymbiotic hypothesis with respect to its namesake organelle. In humans, it contains 37 known genes, most coding for tRNA's. FTP, name this genetic material generally inherited only for themother; a ribonucleic acid present in the "power plant" of cells.
Answer: mitochondrial deoxyribonucleic acid (prompt on deoxyribonucleic acid, mitochondria or mitochondrion) A prolonged fast can activate a selective pathway in these structures that involves KFERQ proteins. I-cell disease resultsfrom a defect in the addition of the mannose-6-phosphate moiety used to target enzymes to them, and a deficiency ofthe enzyme glucocerebrosidase ("gloo-coh-ser-eh-BRO-sih-dase") in these organelles leads to Gaucher's ("go-SHAYZ")disease. Their membranes contain hydrogen ion pumps and chloride ion channel proteins that maintain an interior pHof about 4.8, ideal for their proteasomes and other hydrolytic enzymes. Autophagy occurs in, FTP, these organellesresponsible for enzymatic digestion of cell components and ingested matter.
Answer: lysosomes Defective versions of these can result in RCPD, AMN, and X-linked adrenoleukodystrophy. Another defect results in theinability to metabolize phytanic acid, a condition known as Refsum disease, and it is also the location whereplasmalogen synthesis begins. Some proteins being targeted to this organelle rely on a string of serine-lysine-leucineresidues at the C-terminus, the SLK sequence. Those amino acids bind to PTSR receptors, defects in which can causeZellweger syndrome. Fatty acids are oxidized to acetyl CoA here, though it is best known for the enzyme catalase. For10 points, name this organelle that isolates and degrades toxic peroxide species.
Answer: peroxisomes Noncanonical functions for this structure are regulation of telomerase, modification of microRNAs, interaction with viralproteins, and sequestration of proteins important for tumor suppression and the cell cycle. Consisting of fibrillarcenters, dense fibrillar components, and granular components, it is surrounded by a layer of condensed chromatin.
Disappearing totally during mitosis, this structure is the location of 75% of the polymerase activity in the cell, primarilythrough the transcription of rDNA through RNA polymerase I. Also the location of synthesis of tRNAs and signalrecognition particles, FTP, name this cellular organelle that is the primary site of ribosome synthesis, a subcomponent ofthe nucleus.
Answer: nucleolus Defects in mannose phosphorylation lead to their failure and I-cell disease, characterized by inclusion bodies similar tothose seen in Tay-Sachs disease. In excess, Vitamin A can induce hepatotoxicity by weakening the membrane of theseorganelles in the liver. This break in their membrane releases hydrolytic enzymes, which cause the death of the cell.
FTP, name these organelles active during apoptosis, which act as the digestive system of the cell.
Answer: Lysosomes One subclass of this organelle features the microsomal P450 oxidase system, which plays a key role in the first-passeffect through the liver. Another variation of this organelle contains the protein calsequestrin, important in regulatingskeletal muscle contraction. In neurons this organelle produces many neurotransmitters, stains purple when exposed toaniline, and thus is referred to as Nissl substance. Its most famous functions, however, are either lipid synthesis orprotein production. FTP, name this organelle continuous with the nuclear membrane that comes in sarcoplasmic,smooth, and rough varieties.
Answer: endoplasmic reticulum [accept smooth endoplasmic reticulum before "calsequestrin"] Techniques used to study this process involve detecting the breakdown of the LC3 protein, localization of LC3 toeponymous bodies, and direct identification of these bodies by their characteristic double-membrane form.
Potentiators of this process include rapamycin, which induces formation of complexes of Atg proteins critical forinduction of it. Too much of it can cause type II programmed cell death, although in cancers it can also promote cellularsurvival. Primarily induced in response to low nutrient levels, FTP, name this cellular process in which old organelles aresurrounded by a membrane and fused to lysosomes for recycling, which takes its name from the Latin for "self-eating." Answer: autophagy (accept type II programmed cell death before it is mentioned) Proteins destined for this structure are tagged at their N-linked oligosaccharides by mannose-6-phosphate, which allowreceptors to mediate vesicular transport of tagged proteins. Malfunction of this process is the cause of inclusion celldisease, while the lack of a critical enzyme inside this organelle leads to diseases such as Pompe's disease and Hurlersyndrome. The lack of hexos-aminidase A causing buildup of gangliosides in this organelle is the cause of Tay-Sachsdisease. Proton pumps and ion channels in this organelle maintain the pH of 4.8 that is critical for enzymatic function.
Discovered in 1949 by Christian de Duve, FTP, name these organelles that destroy cellular waste and aid in thebreakdown of macromolecules.
Answer: lysosomes While its exact method of attachment and entry are unknown, it is thought to bind to the host cell via MSP-1. Theseorganisms are known for giving rise to a series of apical organelles throughout their life cycles, including the microneme,the rhoptry, and the dense granule. A member of phylum Apicomplexa, it modifies the erythrocyte by exportingproteins such as PfEMP1 on the host surface. Containing 14 chromosomes, 1 mitochondrion, and 1 plasmid, species inthis genus include falciparum, vivax, and ovale, and it changes form from a merozoite to a trophozoite, and then to asporozoite. FTP, identify this genus carried by the Anopheles mosquito, the agent that causes malaria.
Answer: Plasmodium Leber optic neuropathy was the first disease to be associated with this organelle, while Kearns Sayre syndrome iscaused by defects in these which leads to Ophthalmoparesis and ptosis. The Tom and Tim proteins are involved withtranslocation of proteins across their membranes. Cytochrome C is also found within these structures, and is releasedinto the cell to trigger apoptosis. These organelles have two membrane systems with the inner one possessing foldscalled cristae, and they possess their own genome which is used to analyze matrilineal ancestry. For 10 points, namethis organelle, often referred to as the "power source" of the cell.
Answer: mitochondrion [or mitochondria] One type of bodies found in these were identified by the fusion of RAR alpha with PML proteins in promyelocyticleukemia patients. Pores in its membrane are made of proteins rich in phenylalanine and glycine residues, and importinto and export out of this structure requires karyopherins, and is regulated by Ran proteins. Cajal bodies are also foundin this organelle, which can be visualized by Hoechst or DAPI staining. The rough endoplasmic reticulum is continuouswith its double membrane, and it is the location of mRNA splicing as well as DNA transcription. For 10 points, name thisorganelle which contains chromatin that is only found in eukaryotes.
Answer: nucleus The flgB ["f-l-g-b"] operon controls transcription of the distal rod protein of its blepharoplast, and fliF proteins aggregateinto the MS ring of this structure. The secretion system referred to as T3SS uses a simpler version of one component ofthis organelle. Nexin links the components of these organelles together, preventing them from being dissolved, while itsshaft passes through four rings, rather than two, in Gram-negative bacteria. In eukaryotes their motion results from thealternating movements of two sets of dynein arms, while those of prokaryotes are screw-shaped and rotate. Thedefining external feature of sperm cells, these are, for 10 points, what long structures in eukaryotes that whip back andforward to allow for movement?Answer: flagellum [or flagella] HSP90 aids in the delivery of substances to the translocase of outer membrane complex found on these entities, whichare also the site of the combination of protoporphyrin IX with iron to form heme. Kearns-Sayre syndrome and Leber'shereditary optic neuropathy are examples of diseases associated with the transfer of their genetic material, a processwhich in over 99 percent of mammals involves maternal inheritance as there are roughly 1000 times more of these inoocytes than in sperm. Along with chloroplasts, these are the subjects of endosymbiotic theory, and the foldings of its inner membrane are known as cristae. FTP, identify this double-membrane cell organelle that generates the majority ofthe ATP in a cell.
Answer: mitochondria One disorder involving this organelle is caused by a mutation in E325K on the NAGA gene, called Schindler disease. Onemethod of transporting molecules to it involves an hsc70-containing chaperone complex. Another disorder results inenzyme transport to the extracellular space rather than this organelle and is called I-cell disease. Proteins are markedfor this structure with mannose-6-phosphate, and proton pump activity in this organelle maintains the acidic pHnecessary for its primary function. One genetic defect results in ganglioside buildup in this organelle and thus causesTay-Sachs disease. For 10 points, identify this cellular organelle that breaks down macromolecules with the aid of acidhydrolases.
Answer: lysosome Proteins destined for these structures often possess a C-terminal SKL sequence. Mutations in the ABCD1 gene lead toa demyelination disease involving these organelles called adrenoleukodystrophy. In humans, a reduction in theirnumber is the cause of Zellweger syndrome. Beta oxidation of fatty acids to acetyl CoA occurs in these structures, whichalso contain the enzyme catalase, which is responsible for breaking down a certain toxic material into oxygen andwater. For 10 points, name these organelles that isolate and destroy hydrogen peroxide.
Answer:  peroxisome Chloramphenicol stalls the activity of this structure, while reversal of its primary function is enabled by LepA. Thatfunction is carried out using the GTPase activity of EF-G. One of its main substrates is assembled using the enzyme aaRS,and in prokaryotes, its 16S portion attaches to the Shine-Dalgarno sequence. Designated 70S or 80S, this organelletransfers a nascent peptide from its P-site to its A-site where aminoacyl-tRNA accepts the polymerizing protein chain.
The tRNAs then exit from its E-site. Coming in free and bound varieties, for 10 points, name this organelle sometimesfound on rough ER that contains two subunits and is the site of protein synthesis.
Answer: ribosome In Drosophila, the fuzzy onions gene regulates the fusion of these organelles in sperm, which creates the nebenkern.
One disease that affects these organelles results in a “salt-and-pepper” pigmentation of the retina. In anotherdisease, these organelles take the appearance of “ragged red fibers” under a Gomori trichrome stain. In additionto the aforementioned Kearns-Sayre syndrome, the TIM/TOM complex sees the translocation of proteins into them.
Enzymatic abnormalities in these organelles can result in significant oxidative stress, and cytochrome C is found withinthese structures. It contains inner compartments called cristae. For 10 points, name these organelles with namesakematrilineal DNA, known as the “powerhouse” of the cell.
Answer:  mitochondria [or mitochondrion] Sodium-mediated antiport membrane proteins in these structures can be passively inhibited through the use of digitalisextracts which block sodium-potassium ATPases. They are mechanically coupled to plasma membrane dihydropyridinereceptors. Though not found in neurons, they possess ryanodine receptors which can be activated when phospholipaseC cleaves PIP2 into DAG and the signaling molecule inositol trisphosphate. Depending on the cell type, extensions ofthese structures form dyads or triads with adjacent T-tubules and they contain the protein calsequestrin. For 10 points,name these structures found within muscle cells which store intercellular calcium and are a modified form of anorganelle that is continuous with the nuclear envelope and comes in a rough form.
Answer: sarcoplasmic reticulum [or SR; prompt on endoplasmic reticulum or ER]  When TFEB translocates to the nucleus, it results in a corresponding increase in the number of these organelles. Aglycosphingolipid in this organelle, iGb3, is necessary for the production of natural killer cells. P-type lectins directenzymes that have been tagged with mannose-6-phosphate to this organelle. Christian Rene de Duve discovered this organelle after centrifuging liver cells and discovering an abnormally high amount of acid phosphatase. Malfunction ofthis organelle can lead to a buildup of gangliosides in Tay-Sachs disease. For 10 points, name these organelles thatperform autophagy by digesting unneeded or excess cellular materials.
Answer: lysosomes Proteins targeted for either this structure or the plasma membrane are tagged with the amino acid sequence Y-X-X-phi,where X is any residue and phi is any hydrophobic residue. One protein found here, GAAP, was found in 2009 to reducethe frequency of oscillations of cytosolic calcium concentrations. In plants, hemicelluloses and pectins are created here,and it is location of sphingomyelin synthesis from ceramide. Sulfotransferases sulfate glycosaminoglycans andproteglycans in this structure. Vesicles marked with the COPII ("cop-2") protein transport molecules from endoplasmicreticulum to its cis face. This organelle is composed of flattened discs called cisternae. For 10 points, name this protein-modifying organelle named for an Italian biologist.
Answer: Golgi body [or Golgi apparatus; or Golgi complex] 5. Cycads are some of the only plants to have them, and they are unique to eukaryotes. Like a longer structure, theyhave a 9+2 arrangement of constituent structures which make up the axoneme. The Fallopian tubes use them toconduct eggs from ovary to uterus and the trachea has a lining of them for cleaning purposes. Rotifers have a crown ofthem, which in some species makes them spin like wheels, and several of them are also found on paramecium. For 10points, identify these short organelles which allow many eukaryotic cells to move.
Answer: cilia [or cilium] (HSAPQ1) 4. Their dysfunction may cause Hurler syndrome, which arises from an iduronidase deficiency in these organelles. Asevere form of mucolipidosis, also called i-cell disease, occurs due to improper phosphorylation of a mannose sugar tagapplied to objects destined for them. Better known for failures that cause Tay-Sachs disease, they contain several acidhydrolases which work within their acidic environments. For 10 points, identify these organelles, named for theirfunction of breaking down macromolecules acquired from phagocytosis.
9. Structures found within it include ones discovered by Cajal, which are thought to be involved in the modification ofsnRNPs. Alpha-solenoid and beta-propeller folds can be found in the pores located on the double membrane envelopeof this structure, which is continuous with the rough endoplasmic reticulum. Red blood cells lack this structure, whichcontains protein-DNA complexes called euchromatin and heterochromatin. For 10 points, identify this organelle, whichhouses the cell's genetic information.
Answer: nucleus (HSAPQ1) This entity requires proper functioning of the protein frataxin, which, when mutated, causes Friedrich’s ataxia. Anotherdisease of these bodies limits eye movement and is known as Kearns-Sayre syndrome. Lynn Margulis popularized theidea that these bodies are proteobacteria that underwent endosymbiosis. This organelle’s inner folds are called cristae,and it contains its own DNA that is inherited exclusively from an organism’s mother. For 10 points, name thispowerhouse of eukaryotic cells.
Answer: mitochondria [or mitochondrion] (HSAPQ2) Proteins required for transport of molecules into this structure include the Tic and Toc complexes, and adding twoarginine residues to the N terminus of proteins marks them for one part of this structure. Along with an analogousstructure found in all eukaryotes, this organelle is evidence for the (*) endosymbiotic theory. It consists of stacks ofthylakoids called grana; outside the thylakoids is a space called the stroma in which the Calvin cycle occurs. For 10points, name this organelle responsible for carbon fixation through photosynthesis.
Answer: chloroplasts (HSAPQ3) This organelle is the site of p53 localization due to proteasome inhibition. Spliceosomal RNA is regulated by the smallRNA found here. During mitosis, the components of this structure are synthesized from chromosomal organizer regions,and this structure coalesces from ten smaller structures after cell division. It contains (*) fibrillar centers and theprotein fibrillarin, which is involved in ribosomal RNA processing. For 10 points, name this organelle in whichcomponents of ribosomes are assembled, found within the nucleus.
Answer: nucleolus (HSAPQ3) A disease caused by a defect in these is characterized by progressive limitation of eye movement and is known asKearns-Sayre syndrome. This organelle contains cytochrome c, and, like the nucleus, this organelle has a doublemembrane, with the (*) inner one featuring folds known as cristae. Like the nucleus and chloroplast, they also containtheir own DNA. The site of the Krebs cycle and completion of the electron transport chain, for 10 points, name thisorganelle, the cell’s power source.
Answer: mitochondria [accept mitochondrion] (HSAPQ3) In some organisms, DNA associated with this structure can confer atrazine resistance via the psbA gene. Organisms alsoexpress a family of envelope protein translocases for this organelle called Tic and Toc. That aforementioned type ofDNA often forms trimers and tetramers. Molecules associated with this structure include plastoquinone and NADPH,which is used in this structure’s electron transport chain. Stacks of thylakoids are found dispersed throughout thestroma of this organelle. For 10 points, name this organelle, the main site of photosynthesis.
One structure in this organelle forms in the presence of VIPP1 and contains the cytochrome b6f complex; that structurecontains plastocyanin. The presence of a peptidoglycan cell wall in this organelle in certain glaucophyte algae supportsthe hypothesis that this organelle arose from endosymbiosis with cyanobacteria. This organelle's lamellae connect thegrana, stacks of structures called thylakoids, which sit inside its inner membrane. For 10 points, identify this commonorganelle, whose stroma contains enzymes like rubisco, the site of photosynthesis.
Answer: chloroplast This organelle can malfunction in a disease sometimes called acid maltase deficiency in which it fails to break downglycogen. Another disease associated with this organelle may be treated by letting sialidase metabolize GM2gangliosides. Proteins destined for this organelle are tagged with mannose-6-phosphate. A disease caused by ahexosaminidase A deficiency in this organelle results in a retinal red spot and death by the age of four, Tay-Sachs. Ituses membrane proton pumps to keep its internal pH at ** For 10 points, name these digestive organelles.
This organelle is responsible for the production of chondroitin sulfate, a group that binds to serine residues, as it is thesite of glycosaminoglycan synthesis. Retrograde transport from this organelle requires COPI [cop-one] proteins, and thespiral-shaped protein dynamin pinches vesicles off of it. Called dictyosomes in plants, they consist of stacks calledcisternae. With a cis face opposite the endoplasmic reticulum and a trans face open to the cytoplasm, for 10 points,name this site of macromolecule processing, an organelle named for an Italian scientist.
Answer: Golgi apparatus [or Golgi body; or Golgi complex; prompt on dictyosomes] The abnormal creation of these objects in animal cells due to ineffectiveness of an enzyme in a related structure is amajor cytological symptom of mannosidosis. A series of HRP membrane flow studies have placed good bounds on therate at which these organelles can be created. HLA class II specialization occurs in these organelles after HLA class II isinjected into one (*) containing a degraded antigen. Cytoplasmic tail anchors can be used to target substances to theseorganelles by vesicular transport to create plant bioreactors. In plants these cells are bounded by a membrane knownas a tonoplast, while in animal cells they are usually created by pinocytosis or phagocytosis. Used in plants to generallystore metabolytes and to maintain turgor pressure, FTP, name these organelles which consist of large, water-filled sacs Posttranscriptional insertions and deletions of uridine residues occur in these structures in trypanosomes. Thesestructures are adversely affected in Barth syndrome due to a defect in the biosynthesis of cardiolipin. Other diseasesassociated with them include Friedreich’s ataxia and Kearns-Sayre syndrome. Damage to them results in the formationof a permeability transition pore and can trigger (*) apoptosis through the release of cytochrome c. They may containthermogenin when found in brown fat, and they contain the malate shuttle, which regenerates NADH inside them. FTPname these organelles that contain F0F1 ATP synthase and that are the sites of the citric acid cycle and the electrontransport chain, generating energy for the cell.
Answer: mitochondria or mitochondrion (09Terrapin) Weisberger et al noted that chloramphenicol inhibits the action of this structure in microbial cells, though notmammalian ones, and the antibiotics edeine and pantamycin work by binding to this structure. Its L11 stalk allows forthe interaction with RF-1 that allows release from its substrates, and the structure it forms with its substrates isstabilized by the interaction of its 16S arm with the (*) Shine-Dalgarno sequence in prokaryotes. This entity’s L1 stalkbinds to its deacylated substrate in the P-site, encouraging the ratcheting that brings about translocation of tRNA to theE-site, and this organelle’s production occurs in the nucleolus. FTP, name this cellular organelle, which has two subunitsand is responsible for translation of mRNA into proteins.
Answer: ribosomes (09Terrapin) The flgB ["f-l-g-b"] operon controls transcription of the distal rod protein of its blepharoplast, and fliF proteins aggregateinto the MS ring of this structure. The secretion system referred to as T3SS uses a simpler version of one component ofthis organelle. Nexin links the components of these organelles together, preventing them from being dissolved, while itsshaft passes through four rings, rather than two, in Gram-negative bacteria. In eukaryotes their motion results from thealternating movements of two sets of dynein arms, while those of prokaryotes are screw-shaped and rotate. Thedefining external feature of sperm cells, these are, for 10 points, what long structures in eukaryotes that whip back andforward to allow for movement?Answer: flagellum [or flagella] (09Winter) HSP90 aids in the delivery of substances to the translocase of outer membrane complex found on these entities, whichare also the site of the combination of protoporphyrin IX with iron to form heme. Kearns-Sayre syndrome and Leber’shereditary optic neuropathy are examples of diseases associated with the transfer of their genetic material, a processwhich in over 99 percent of mammals involves maternal inheritance as there are roughly 1000 times more of these inoocytes than in sperm. Along with chloroplasts, these are the subjects of endosymbiotic theory, and the foldings of itsinner membrane are known as cristae. FTP, identify this double-membrane cell organelle that generates the majority ofthe ATP in a cell.
Answer: mitochondria (09Winter) One type of bodies found in these were identified by the fusion of RAR alpha with PML proteins in promyelocyticleukemia patients. Pores in its membrane are made of proteins rich in phenylalanine and glycine residues, and importinto and export out of this structure requires karyopherins, and is regulated by Ran proteins. Cajal bodies are also foundin this organelle, which can be visualized by Hoechst or DAPI staining. The rough endoplasmic reticulum is continuouswith its double membrane, and it is the location of mRNA splicing as well as DNA transcription. For 10 points, name thisorganelle which contains chromatin that is only found in eukaryotes.
Answer: nucleus (08Fall) Leber optic neuropathy was the first disease to be associated with this organelle, while Kearns Sayre syndrome iscaused by defects in these which leads to Ophthalmoparesis and ptosis. The Tom and Tim proteins are involved with translocation of proteins across their membranes. Cytochrome C is also found within these structures, and is releasedinto the cell to trigger apoptosis. These organelles have two membrane systems with the inner one possessing foldscalled cristae, and they possess their own genome which is used to analyze matrilineal ancestry. For 10 points, namethis organelle, often referred to as the “power source” of the cell.
Answer: mitochondrion [or mitochondria] (08Fall) Its residual body can form a lipofuscin pigment granule, and proteins like AP1 and GAAT are necessary for its function. An enlargement of it that causes damage to muscle cells is called Pompe (pom-PAY) disease, while a defect in sugarmetabolism causes it to malfunction in Hurler's syndrome.  The home to V-class pumps, it takes in triskelion shapedclathrin-bound vesicles, and proteins tagged by mannose-6-phosphate are bound for it.  Critical for autolysis, FTP namethis organelle that uses proton pumps to generate an acidic environment where its hydrolases can act to digestmaterial.
Answer: lysosome Proteins destined for these structures often possess a C-terminal SKL sequence. Mutations in the ABCD1 gene lead to ademyelination disease involving these organelles called adrenoleukodystrophy. In humans, a reduction in their numberis the cause of Zellweger syndrome. Beta oxidation of fatty acids to acetyl CoA occurs in these structures, which alsocontain the enzyme catalase, which is responsible for breaking down a certain toxic material into oxygen and water. For10 points, name these organelles that isolate and destroy hydrogen peroxide.
Chloramphenicol stalls the activity of this structure, while reversal of its primary function is enabled by LepA. Thatfunction is carried out using the GTPase activity of EF-G. One of its main substrates is assembled using the enzyme aaRS,and in prokaryotes, its 16S portion attaches to the Shine-Dalgarno sequence. Designated 70S or 80S, this organelletransfers a nascent peptide from its P-site to its A-site where aminoacyl-tRNA accepts the polymerizing protein chain.
The tRNAs then exit from its E-site. Coming in free and bound varieties, for 10 points, name this organelle sometimesfound on rough ER that contains two subunits and is the site of protein synthesis.
Answer: ribosome (09Fall) One disease of this type sees the formation of curved structures called "banana bodies," while one example of this typeof diseases is treated by the drug miglustat. Another disease of this type sees the generalized occurrence of lesionscalled angiokeratomas and is alternatively called angiokeratoma corporis diffusum. In addition to Farber’s disease andFabry's disease, these diseases are commonly associated with patients exhibiting hepatomegaly or splenomegaly. Themost prominent examples of these diseases include ones in which the activities of L-iduronidases are impaired,exemplified by the Hurler syndrome and the Scheie syndrome. Other examples of these diseases include Gaucher'sdisease, which sees improper functioning of beta-glucosidase. For 10 points, identify this class of diseases, whereinmacromolecules get accumulated in a certain organelle, whose other examples include the Tay-Sachs disease.
Answer: lysosomal storage diseases [accept equivalents; prompt on "diseases involving mutations of hydrolases"](09MO)

Source: http://www.texasquizbowl.org/moodle/a/33_Organelles.pdf