Carbon Skeletons Khan Flashcards

1
Q
  1. What is the only tissue that has all the pathways for amino acid synthesis and degradation?
A
  1. Liver
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2
Q
  1. What are all 20 AA’s able be broken down into?
A
  1. AcetylCoA, pyruvate, 4 TCA intermediates (OAA, fumarate, succinylCoA, alphaketoglutarate)
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3
Q
  1. What is a ketogenic AA?
A
  1. Can be made into AcetylCoA or acetoacetate
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4
Q
  1. What is a glucogenic AA?
A
  1. Can be made into glucose
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5
Q
  1. What are the purely ketogenic AA’s?
A
  1. Leucine/Lysine
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6
Q
  1. What are the ketogenic/glucogenic AA’s?
A
  1. Phe/Tyr, Ile, Trp
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7
Q
  1. Describe the pathway of glycine synthesis
A
  1. Pyruvate –> serine –> glycine via PLP and THF –> Gly
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8
Q
  1. During glycine synthesis what is a methyl group transferred to that can form carbon dioxide
    and ammonium ion?
A
  1. THF
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9
Q
  1. By enzymatic action of an oxidase what can glycine become?
A
  1. Glyoxylate
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10
Q
  1. What is the condition caused by defective transaminase in Gly metabolism?
A
  1. Oxaluria type I (due to less conversion of glyoxylate back to Gly and accumulation of
    glyoxylate)
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11
Q
  1. What is a clinical condition manifested by excess glyoxylate
A
  1. kidney stones (ppt. with calcium)
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12
Q
  1. Degradation of what other AA other than Ser can generate Gly?
A
  1. Thr
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13
Q
  1. In cysteine metabolism where does the sulfur originally come from?
A
  1. Met
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14
Q
  1. Met is degraded to generate what product (via methyltransferase)
A
  1. Homocysteine
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15
Q
  1. What intermediate is used in Met degradation for methyl transfer?
A
  1. SAM
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16
Q
  1. What can homocysteine be then converted into?
A
  1. Back to Met or into cysteine via cystathionase
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17
Q
  1. What can cysteine be degraded to?
A
  1. Sulfinic acid, in presence of alpha ketoglutarate it can be degraded to pyruvate (thus it
    is a glucogenic AA)
18
Q
  1. The sulfhydryl groups of cysteine can be oxidized to form what which generate sulfated
    proteoglycans
A
  1. PAPs
19
Q
  1. Can cysteine inhibit cystathione synthase via feedback inhibition?
A
  1. Yes
20
Q
  1. What does a deficiency in cystathionase lead to?
A
  1. Cystathionuria (benign disorder)
21
Q
  1. What could cause a deficiency in cystathione?
A
  1. Dietary deficiency of pyridoxine(Vit. B6) or Vit. B12
22
Q
  1. What does a deficiency in cystathionine synthase cause?
A
  1. Homocystinemia/Homocystinuria (accumulation of homocysteine and met in brain,
    can cause loss in cognitive fx. , tall stature, CV disease, thrombi, emboli, etc.)
23
Q
  1. What is the treatment for homocystinuria?
A
  1. Low met diet, high doses of pyridoxine
24
Q
  1. Can our bodies synthesize aromatic rings?
A
  1. No (makes them essential AA’s)
25
Q
  1. How is tyrosine synthesized?
A
  1. From Phe via hydroxylation reaction using oxygen and tetrahydrobiopterin
26
Q
  1. T or F: Any defective step in Phe or Tyr synthesis causes severe diseases
A
  1. T
27
Q
  1. What does a deficiency in Phe hydroxylase present as?
A
  1. Phenylketouia (PKU), phenylpyruvate accumulates and is excreted (mental retardation, lighter skin due to impaired tyrosine synthesis)
28
Q
  1. What is the treatment for PKU?
A
  1. Diet low in Phe, rich in Tyr
29
Q
  1. What is the first step in degradation of tyrosine?
A
  1. Removal of ammonia via tyrosine aminotransferase
30
Q
  1. What does a defect in this step cause?
A
  1. Tyrosinemia II (eye, skin, lesions, neurological disorders)low tyr and low phe diet is
    treatment
31
Q
  1. What does deficiency in homogentisate oxidase cause?
A
  1. Alcaptonuria (accumulation of homogentisate which causes arthritis)
32
Q
  1. What does a deficiency in degradation of fumarylacetoacetate cause?
A
  1. Tyrosinemia I (liver failure and death within 1 yr of life)
33
Q
  1. Where does the aromatic ring of Trp end up via degradation pathways?
A
  1. NAD/NADP
34
Q
  1. Where does the carbon skeleton of Trp end up?
A
  1. Ala
35
Q
  1. Trp to NAD requires what cofactor? What does a deficiency in this cofactor cause?
A
  1. PLP, pellagra like symptoms
36
Q
  1. What are the branched chain AA’s?
A
  1. Val, Ile, Leu
37
Q
  1. What can these AA’s generate via transamination rxn?
A
  1. Alphaketovalerate, alpha keto beta methylvalerate, alphaketoisocaproate
38
Q
  1. What are branched chain AA’s ultimately converted to?
A
  1. propionylCoa or Acetyl CoA
39
Q
  1. What is homocistinuria type 1 caused by?
A
  1. cystathionine synthase deficiency (high homocystine and methionine levels)
40
Q
  1. What is homocystinuria type 2/3 caused by?
A
  1. deficiency in methl‐b‐12 and methyl THF synthesis (high serum homocysteine and low
    methionine levels)