Term
| An essential Amino Acid for normal individuals |
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Definition
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Term
| The carbon chain for Cys comes from |
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Definition
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Term
When muscle is used as a source of energy and
AA’s, amino groups are transported to the liver as |
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Definition
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Term
| The synthesis of each molecule of urea requires ATP |
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Definition
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Term
| The TCA Cycle intermediate produced by the urea cycle is... |
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Definition
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Term
| A deficiency of Ornithine Transcarbamylase |
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Definition
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Term
| A deficiency of Cytathionine Synthetase results |
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Definition
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Term
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Definition
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Term
| Trypsin cleaves specifically for ___, ___ amino acids. |
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Definition
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Term
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Definition
| aromatic AA(Phe, Tyr, Trp, Leu) |
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Term
| enterokinase (enteropeptidase) activates ___ to ___ (which, in turn, activates ___, ___, ___) |
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Definition
trypsinogen; trypsin; chymotrypsinogen, proelastase
and procarboxypeptidase |
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Term
| ___ --> ___ autoactivation occurs at acid pH with Asp in active site |
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Definition
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Term
| 1/3 AA pool from ___ and 2/3 AA pool from ___. AA Transport: AA’s (L/D amino acids) are principally transported via ___ into the cytosol of intestinal epithelial cells and into the blood via ___ transporters. |
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Definition
| diet, turnover; L-aa; Na+-dependent co-transporters; facilitated |
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Term
| AMINO ACID TRANSPORT: cystine not reabsorebed from urine and precipitates in kidneys |
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Definition
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Term
| AMINO ACID TRANSPORT: failure to absorb Trp in intestines or recover from urine-results in pellagra b/c Trp def leads to niacin def |
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Definition
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Term
| Oxidative deamination releases ___ from AAs, the classic example is ___ -> alpha-KG and NH4+, catalyzed by glutamate dehydrogenase. This easily reversible reaction uses ___ or ___ as a coenzyme. |
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Definition
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Term
| transaminase/amino transfer requires _________ coenzyme |
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Definition
| pyridoxal phosphate (Vit.B6) |
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Term
| Plays role in transfer of methyl group from 5-methyl THF to Homocysteine to make Met. |
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Definition
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Term
| recieve 1-C groups in degradative steps and donate them in synthesis steps |
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Definition
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Term
| cofactor in conversion of methylmalonyl-CoA to Succinyl-CoA by methylmalonyl mutase and conversion of homocysteine to methionine by methionine synthase...deficiency leads to anemia |
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Definition
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Term
| Thr enters as ___. Ile & Val enter as ___. |
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Definition
| alpha-Ketobutyrate; Propionyl CoA |
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Term
| A Defect in methylmalonyl CoA mutase(which requires ___) leads to ___ disease in the conversion of homocysteine to methionine. |
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Definition
| Vit. B12; Methylmalonic acidemia |
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Term
| 3-C AAs, Gly, Ala, Ser, Cys(all degrade to pyruvate and thus are glucogenic/gluconeogenic) -> |
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Definition
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Term
4-C AAs: Asp, Asn--> ___.
Met --> ___ --> ___. Genetic defects here result in ___ and ___. Sulfur used for Cys synthesis from ___. |
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Definition
Oxaloacetate.
SAM, Succinyl CoA; Homocystinuria and Cystathioninuria; Ser |
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Term
| Ile and Val enter as _________. |
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Definition
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Term
| ___________ required for conversion of propionyl CoA --> Methylmalonyl CoA |
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Definition
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Term
| _________ required for conversion of methylmalonyl CoA --> Succinyl CoA |
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Definition
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Term
| 5-C AAs His, Arg, Pro, Glu, Gln --> _________. |
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Definition
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Term
| His --> ________ or ___________. |
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Definition
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Term
| Pro --> Glu via _________ . |
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Definition
| pyyroline 5-carboxylate intermediate |
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Term
| Arg & Pro --> _________ --> __________. |
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Definition
| Glutamate gamma-semialdehyde, glu |
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Term
| Branched chain aa: Ile and Leu primarily metabolized in ___, Val in ___. Val & Ile --> ___ (like Met). |
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Definition
| muscle; brain; Succinyl CoA |
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Term
| defective branched chain alpha-ketoacid decarboxylase |
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Definition
| maple syrup urine disease |
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Term
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Definition
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Term
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Definition
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Term
| glucogenic, non-essential AAs that degrade to pyruvate are ________, ________, _________, and _________. |
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Definition
| Ser, Gly, Cys, Ala (some guys cick ass) |
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Term
| The AAs are gluconeogenic and degrade to TCA cycle intermediates- _______ and _______. |
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Definition
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Term
| Thr --> ___ -> ___ -> ___. |
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Definition
| alpha ketobutyrate, propionyl CoA, Succinyl CoA. |
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Term
| ___ and ___ pathways converge at alpha-ketobutyrate and metabolic defects here include ___ and ___. |
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Definition
| Met, Thr, Propionic Acidemia, Methylmalonic Acidemia (Ile and Val enter here as propionyl CoA) |
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Term
| Aromatic AAs- Phe --> ___ and with defective Phe hydroxylase you get the condition known as ___ (PKU -> -> phenylacetate). ___ is needed for all hydroxylations. |
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Definition
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Term
| Tyr --> ________ --> __________, _________ (catecholamines) |
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Definition
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Term
| Condition due to missing tyrosinase that converts DOPA to melanin |
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Definition
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Term
| When Tyr -X-> Fumarate and dark urine is produced due to oxidation of homogentisate, the condition is called... |
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Definition
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Term
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Definition
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Term
| Essential AAs - Branched Chain |
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Definition
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Term
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Definition
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Term
| Essential AAs - Sulfur containing |
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Definition
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Term
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Definition
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Term
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Definition
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Term
| Non essential AAs-Pyruvate--> |
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Definition
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Term
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Definition
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Term
| Non essential AAs-Alpha-ketoglutarate--> |
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Definition
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Term
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Definition
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Term
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Definition
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Term
| Non essential AAs-CO2 & NH4--> ______ --> ______ . |
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Definition
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Term
| NE AAs-3-Phosphoglycerate--> |
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Definition
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Term
| urea synth begins in the... |
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Definition
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Term
| Urea Cycle: First is synth of ________ from NH4+, CO2, And 2-ATP using __________. |
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Definition
| carbamoyl phosphate, carbamoyl synthase I (with N-acetyl-glutamate as its positive allosteric modulator) |
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Term
| Ornithine transcarbamylase forms ________ from Orn and Carbamoyl Phosphate. |
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Definition
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Term
| citrulline is transported into the _________ where it is converted to __________. |
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Definition
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Term
| Arginosuccinate lyase cleaves arginosuccinate to ________ and ________. |
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Definition
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Term
| Arginase, primarily found in ________, converts Arg to ________ + ________; arginase deficiency results in ________. |
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Definition
| liver, urea, orn; arginemia |
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Term
| Urea synth requires ________ equivalents of ATP |
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Definition
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Term
| Irreversible steps, the first and last are catalyzed by _______ and ________. |
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Definition
| carbamoyl synthetase, arginase |
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Term
| Patient can't synth urea, dies due to incr NH4+ |
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Definition
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Term
| Other metabolic defects include _________, __________, and ________. |
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Definition
| Citrullinuria, Arginosuccinic acidemia, and Arginemia (all cause retardation) |
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Term
| ______ and _______ are used to remove ammonia and treat Hyperammonemias via conjugation to Gly and Gln, respectively |
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Definition
| Benzoic Acid, Phenyl Acetate |
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Term
| Gly and Arg --> ______, ______, and _____. |
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Definition
| Creatine, Creatine Phosphate(energy source in mm) and Creatinine |
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Term
| Arg is substrate for the synth of -> ___ |
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Definition
NO (smooth mm relaxation; it activates guanylate cyclase to produce cyclic GMP, and cGMP, like cAMP activates a
protein kinase that phosphorylates and inactivates myosin light-chain kinase (MLCK).) |
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Term
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Definition
| Cystinuria, Hartnups Disease, 5-Oxoprolinuria |
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Term
| Enzyme defect in carbamoyl PO4 synthase or N-acetyl glu synthase -> ___. Defect in Orn Transcarbymlase -> ___. |
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Definition
| Type I Hyperammonemia; Type II Hyperammonemia |
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Term
| Defect in Arginosuccinate synthase -> ___, defect in arginosuccinate lyase -> ___, defect in arginase -> ___. |
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Definition
| citrullinuria; arginosuccinic acidemia; arginemia |
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Term
| Defect in Trp transport -> ___; Defect in dibasic aa transport -> ___; Defect in Phe Hydroxylase or H4 Biopterin Synthase. |
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Definition
| Hartnup's w/ niacin def (pellagra); Cystinuria; PKU |
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Term
| Defect in homogentisate oxidase -> ___; Defect in tyrosinase -> ___; Defect in cystathione synthase -> ___. |
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Definition
| alkaptonuria(black piss); albinism; homocystinuria; |
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Term
| Defect in methylmalonic CoA Mutase -> ___; Defect in decarboxylase -> ___ disease. |
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Definition
| methylmalonic acidemia; maple syrup urine |
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Term
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Definition
| accept, donate 1-methyl groups |
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Term
| C-3, glycine, alanine, ser, cys-> |
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Definition
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Term
| liver problems lead to ___ metabolism problems |
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Definition
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Term
| The ___ is a large proteolytic complex that degrades proteins marked for destruction by having ___ linked to the epsilon-amino groups of Lys residues via an ATP requiring process |
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Definition
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