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23 Cards in this Set

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D-glucose → Glucose-6-phosphate
Hexokinase/glucokinase (in liver).

(-)Glucose-6-P
Fructose-6-P → Fructose-1,6-BP
Phosphofructokinase-1

Rate limiting step.
Phosphoenolpyruvate → Pyruvate
Pyruvate Kinase
Pyruvate → Acetyl-CoA
Pyruvate Dehydrogenase Complex.

Contains 3 enzymes that require 5 cofactors:
Pyrophosphate (B1), FAD (B2), NAD (B3), CoA (B5), Lipoic Acid.
Pyruvate dehydrogenase deficiency?
Causes backup of substrate resulting in lactic acidosis. can be seen in alcoholics due to B1 deficiency.
Pyruvate metabolism end up as?

[4 end products]
Alanine (ALT)
Lactate (LDH)
Oxaloacetate (Pyruvate carboxylase)
Acetyl-CoA (PDH)
Pyruvate → Oxaloacetate.

[in mitochondria]
Pyruvate carboxylase.

Requires biotin, ATP.
Activated by acetyl-CoA.
Oxaloacetate → Phosphoenolpyruvate.

[in cytosol]
PEP carboxykinase.

Requires GTP.
Fructose-1,6-bisphosphate → Fructose-6-P.

[in cytosol]
Frutose-1,6-bisphosphatase.
Glucose-6-P → glucose.

[in cytosol]
Glucose-6-phosphatase.
Produces ribose-5-P from G6P for nucleotide synthesis?
HMP Shunt.
Glucose 6-phosphate dehydrogenase is rate limiting step.
Deficiency more prevalent in blacks.
Hereditary deficiency of aldose B.
Fructose intolerance.

Sx: hypoglycemia, jaundice, cirrhosis, vomiting.
Defect in fructokinase.
Essential fructosuria.

Benign and asymptomatic condition. Fructose in blood and urine.
Absence of galactose-1-phosphate uridyltransferase?
Galactosemia.
Autosomal recessive.
Damage done by accumulation of toxic substances.
Cataracts, hepatosplenomegaly, mental retardation.
Galactokinase deficiency?
Causes galactosemia and galactosuria.
Lactose intolerant?
Lactase deficiency.

Have bloating, cramps, osmotic diarrhea.
Decreased/absent phenylalanine hydroxylase?
PKU.
Mental retardation, growth retardation, fair skin, eczema, musty body odor.
Deficieny of homogentistic acid oxidase.
Alkaptonuria.
Urine turns black on standing.
Connective tissue dark.
Benign disease.
Debilitating arthralgias possible.
No tyrosinase/defective tyrosine transporters.

Can result from lack of migration of neural crest cells.
Albinism.
Blocked degradation of branched amino acids.
Maple Syrup urine disease.

Ile, Val, Leu.
Have severe CNS defects, mental retardation, and death.
Deficiency of cystathionine synthase.
Methionine synthase deficiency.
Homocystinuria.
Cysteine becomes essential.
Can cause mental retardation, osteoporosis, tall stature, kyphosis, lens subluxation, and artherosclerosis.
Adenosine deaminase deficiency.
SCID.
Absence of HGPRTase.
Lesch-Nyhan Syndrome.

Retardation, self-mutilation, aggression, excess uric acid production.