Carbohydrate Metabolism Flashcards

1
Q

Low Km and Low Vmax; inhibited by G6P

A

Hexokinase

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2
Q

High Km and high Vmax; inhibited by F6P

A

Glucokinase

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3
Q

Inhibitors of PK (pyruvate kinase)

A

ATP, Glucagon, Alanine

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4
Q

Stimulators of PK (pyruvate kinase)

A

Insulin, F1-6 BP; dephosphorylation

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5
Q

Defects in glycolytic enzymes (such as PK, phosphoglucoisomerase and triosephosphate isomerase) malfunction of ATP pumps, intracellular NA+ concentration increases, cell swells, and bursts

A

Hemolytic anemia

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6
Q

Malfunction of GLUT 2; Impaired transport of fructose, glucose and galactose; fasting hypoglycemia

A

Fanconi Bickel

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7
Q

Deficiency in PFK 1 (rate limiting enzyme of glycolysis)

A

Tauri Disease

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8
Q

Rate limiting enzyme of gluconeogenesis

A

Fructose 1, 6 Bisphosphatase

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9
Q

Steps in Gluconeogenesis different from glycolysis

A

Pyruvate-Phosphoenolpyruvate (Pyruvate Carboxylase) and (PEPCK) OAA to PEP
Fructose 1,6 BP-Fructose 6P (Fructose 1,6 Bisphosphatase)
Glucose 6 Phosphate-Glucose (Glucose 6 Phosphatase)

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10
Q

Positive enzymatic regulators of gluconeogenesis

A

Malate Dehydrogenase; PEPCK, Pyruvate carboxylase, and fructose 1,6 bisphosphatase, glucose 6 phosphatase

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11
Q

Positive regulators of gluconeogenesis

A

Citrate, cortisol, thyroxine, acetyl coA

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12
Q

Negative regulators of gluconeogenesis

A

ADP, AMP, Fru 2-6 BP

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13
Q

Fructose 1,6 BP Deficiency

A

Can’t convert Fructose 1,6 BP to Fructose 6 Phosphate
Hypoglycemia
Lactic acidosis
Ketosis
Hyperventilation after artificial sweeteners

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14
Q

Deficiency in glucose 6 phosphatase

Fasting hypoglycemia

A

Von Gierke Disease

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15
Q

Fructose Metabolism

A

Usually used for fatty acid synthesis

Converted to sorbitol (sorbitol dehydrogenase) and to glucose (aldol reductase)-used for glycolysis

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16
Q

Galactose metabolism

A

GALT-rate limiting enzyme (converts Galactose 1 phosphate to Glucose 1 phosphate); in liver can convert to glucose 6 phosphate

17
Q

Deficiency in GALT; can cause cataracts

A

Galactosemia

18
Q

Regenerates glutathione (important in detoxifying H2O2)

A

NADPH (made from PPP)

19
Q

Inhibits glycogen breakdown

A

Insulin, GLUT 4, PKB, GSK3

20
Q

Stimulates glycogen breakdown

A

G protein, cAMP, PKA, PP1, PPK

21
Q

Defective glycogen synthase

A

GSD0

22
Q

Defective acid maltase

Can’t lysosomally degrade glycogen

A

Pompe (GSD II

23
Q

Deficiency in debranching enzyme

Light hypoglycemia and hepatomegaly

A

GSD III/ CORI

24
Q

Deficient branching enzyme
Cirrhosis
Death by age 5

A

GSD IV/ Andersen

25
Q

Deficiency in muscle glycogen phosphorylase

Exercise intolerance

A

GSD V/ MCARDLE

26
Q

Deficiency in liver glycogen phosphorylase

A

GSD VI/ HERs