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23 Cards in this Set
- Front
- Back
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D-glucose → Glucose-6-phosphate
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Hexokinase/glucokinase (in liver).
(-)Glucose-6-P |
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Fructose-6-P → Fructose-1,6-BP
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Phosphofructokinase-1
Rate limiting step. |
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Phosphoenolpyruvate → Pyruvate
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Pyruvate Kinase
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Pyruvate → Acetyl-CoA
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Pyruvate Dehydrogenase Complex.
Contains 3 enzymes that require 5 cofactors: Pyrophosphate (B1), FAD (B2), NAD (B3), CoA (B5), Lipoic Acid. |
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Pyruvate dehydrogenase deficiency?
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Causes backup of substrate resulting in lactic acidosis. can be seen in alcoholics due to B1 deficiency.
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Pyruvate metabolism end up as?
[4 end products] |
Alanine (ALT)
Lactate (LDH) Oxaloacetate (Pyruvate carboxylase) Acetyl-CoA (PDH) |
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Pyruvate → Oxaloacetate.
[in mitochondria] |
Pyruvate carboxylase.
Requires biotin, ATP. Activated by acetyl-CoA. |
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Oxaloacetate → Phosphoenolpyruvate.
[in cytosol] |
PEP carboxykinase.
Requires GTP. |
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Fructose-1,6-bisphosphate → Fructose-6-P.
[in cytosol] |
Frutose-1,6-bisphosphatase.
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Glucose-6-P → glucose.
[in cytosol] |
Glucose-6-phosphatase.
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Produces ribose-5-P from G6P for nucleotide synthesis?
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HMP Shunt.
Glucose 6-phosphate dehydrogenase is rate limiting step. Deficiency more prevalent in blacks. |
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Hereditary deficiency of aldose B.
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Fructose intolerance.
Sx: hypoglycemia, jaundice, cirrhosis, vomiting. |
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Defect in fructokinase.
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Essential fructosuria.
Benign and asymptomatic condition. Fructose in blood and urine. |
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Absence of galactose-1-phosphate uridyltransferase?
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Galactosemia.
Autosomal recessive. Damage done by accumulation of toxic substances. Cataracts, hepatosplenomegaly, mental retardation. |
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Galactokinase deficiency?
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Causes galactosemia and galactosuria.
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Lactose intolerant?
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Lactase deficiency.
Have bloating, cramps, osmotic diarrhea. |
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Decreased/absent phenylalanine hydroxylase?
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PKU.
Mental retardation, growth retardation, fair skin, eczema, musty body odor. |
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Deficieny of homogentistic acid oxidase.
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Alkaptonuria.
Urine turns black on standing. Connective tissue dark. Benign disease. Debilitating arthralgias possible. |
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No tyrosinase/defective tyrosine transporters.
Can result from lack of migration of neural crest cells. |
Albinism.
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Blocked degradation of branched amino acids.
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Maple Syrup urine disease.
Ile, Val, Leu. Have severe CNS defects, mental retardation, and death. |
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Deficiency of cystathionine synthase.
Methionine synthase deficiency. |
Homocystinuria.
Cysteine becomes essential. Can cause mental retardation, osteoporosis, tall stature, kyphosis, lens subluxation, and artherosclerosis. |
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Adenosine deaminase deficiency.
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SCID.
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Absence of HGPRTase.
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Lesch-Nyhan Syndrome.
Retardation, self-mutilation, aggression, excess uric acid production. |
