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31 Cards in this Set
- Front
- Back
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Kwashiorkor is Vit __ deficiency. Some symptoms of this disease include...
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B deficiency
edema, swollen abdomen, diarrhea, peeling skin, vitiligo (white skin spots), low muscle mass...lethargy, apathy |
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Cystinuria is a defect in transport of ___, with a ___ ___ inheritance pattern.
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C - cysteine (SLC3A1, SLC7A9)
O - ornithine R - arginine K - lysine Autosomal Recessive |
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Cystinuria symptoms include:
Treatment? |
kidney stones - precipitation of cysteine and causes bacterial infection
Treated w/ oral hydration, penicillamine, tiopronin, captoril (keep cysteine in solution) |
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Fasting -> Deamination
Steps 1 & 2 |
1. transfer of a-amino via ALT & AST onto glutamate
2. oxidative deamination of product via GDH (glutamate dehydrogenase) |
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name aminotransferases and coenzyme they require
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ALT (alanine-> pyruvate)
AST (aspartate -> oxaloacetate) a-ketoglutarate accepts to become glutamate pyridoxal phosphate markers for: liver disease, MI, muscle disorder |
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pyridoxal phosphate is a ___ derivative
it is used as a coenzyme for |
Vit B6
ALT & AST |
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Transport pathway for ammonia -> liver
(most tissue) |
In tissue: Glutamate (glutamine s
synthase) -> Glutamine In liver: Glutamine..(glutaminase) -> Glutamate..(GDH) -> NH3 -> urea |
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Transport pathway for ammonia -> liver
(muscle) |
Alanine..(ALT)-> Pyruvate ->Glucose -> Pyruvate..(ALT)
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Glutamate dehydrogenase is used to achieve ____, and uses ___ or ___ as coenzymes. It is allosterically activated/inhibited by ___/___
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reversible transamination
(glutamate<-> a-ketoglutarate) NAD+, NADPH ADP/GTP |
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amino acids that can form glutamate
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arginine
histidine proline |
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Fates of urea
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transported in blood to kidney-> urine
diffuses into intestine, cleaved by bacterial urease (Co2 + NH3) ammonia -> feces OR reabsorbed in blood |
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Hyperammonemia is kidney failure due to high ammonia production by ___
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bacterial urease in the inestine
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metabolic acidosis
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in prolonged starvation & diabetes mellitus (blood pH<7.35)
kidney is high in NH3, which reacts w/H+ to get excretable ammonium inability to make NH3 = fatal |
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metabolic alkalosis
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kidney is low in NH3 production
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exclusively ketogenic amino acids
(CO2) produced |
leucine & lysine
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Ketogenic amino acids yield formation of which 3 products
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acetoacetate, acetylCoA, acetoacetylCoA
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glucogenic amino acid catabolism yields...
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glucose, duh
forms pyruvate, & TCA intermediates: a-ketoglutarate, Succinyl CoA, Fumarate, Oxaloacetate |
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Branched-chain amino acids?
Metabolized by? Where? |
Leucine, Valine, Isoleucine
Branched chain a-ketoacid dehydrogenase (BCKD) oxidatively decarboxylates Muscle! |
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MSUD
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Maple Syrup Urine Disease
deficiency in BCKD = buildup of leucine, Valine, Isoleucine |
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Succinyl CoA formed from ____, using which VitB derivative?
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methylmalonylCoA, using 5'deoxyadenylcobalamine - Vit B12 derivative
(VitB12 deficiency = accumulation of Methylmalonyl CoA) |
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carbon skeletons of these 3 amino acids are donted to glutamate to form a-ketoglutarate...
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histidine, arginine, proline
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2 Single-Carbon Carriers
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SAM - S-adenosyl methonine
THF - tetrahydrofolic acid |
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SAM
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formed from Methionine (SAM synthetase)
Carbon carrier hydrolyzed to Homocysteine |
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2 fates of Homocysteine
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1. -> Methonine using Methylcobalamine (B12) & methyl tetrahydrofolate
2. ->..(cystathione synthase) Cystathione -> (Vit B6)..Cysteine |
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THF
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Tetrahydrofolate
carbon carrier (from Histidine, Glycine, Serine) important for formation of methionine |
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Essential amino acids
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Phenylalanine
Valine Threonine Tryptophan Isoleucine Methionine Histidine Arginine Leucine Lysine |
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Steatorrhea
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abnormal protein digestion
lipids & undigested protein in feces deficiency in pancreatic secretion- pancreatitis, cystic fibrosis, removal of pancreas |
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vitamins essential for a.a. metabolism?
2 enzymes responsible for deamination 2 single carbon carriers |
B6 & B12
ALT, AST THF, SAM |
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a.a whose metabolic intermediate serves as a marker for folic acid deficiency
a.a. whose blood levels decrease in cystinuria |
Methionine
CORK - cysteine, ornithine, arginine, lysine |
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2 a.a that generate free ammonium
two fates of urea |
1. glutamine (via glutaminase)
2. asparagine 1. excretion in urine 2. reabsorption in intestine to maintain ammonia (acid-base) balance |
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Patient with undetectable citrulline levels, mild alkalosis, and elevated orotic acid may have this deficiency
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Ornithine Transcarbamylase I deficiency
treatment: sodium benzoate + ARGININE! |
