Metabolism of Other Carbohydrates Flashcards

1
Q

sucrose components

A

fructose + glucose

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2
Q

lactose components

A

glucose + galactose

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3
Q

maltose components

A

glucose + glucose

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4
Q

beta-fructosidase

A

breaks sucrose into fructose + glucose

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5
Q

SLGT1

A

symporter for glucose and galactose into cell

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6
Q

GLUT5

A

channel for fructose

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7
Q

How does fructose enter glycolysis to be metabolized to glucose?

A

fructose –> fructose-1-P (fructokinase) –> DHAP by aldolase-B –> DHAP can not enter the glycolysis pathway

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8
Q

essential fructosuria

A

fructokinase deficiency which results in high fructose levels in urine; no treatment needed

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9
Q

hereditary fructose intolerance

A

aldolase B deficiency (liver) which results in poor feeding, irritability, neonatal jaundice, vomiting, sleepiness, intolerance to fruit

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10
Q

What happens when you have an aldolase B deficiency?

A

inhibits glycolysis and gluconeogenesis and depletes the ATP pool; will lead to accumulation of fructose-1-P

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11
Q

inhibition of gluconeogenesis

A

leads to lactic acidosis

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12
Q

accumulation of fructose-1-P

A

depletes the ATP pool and leads to hyperuricemia (AMP converted to uric acid)

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13
Q

polyol (sorbitol) pathway

A

glucose –> sorbitol –> fructose

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14
Q

aldol reductase

A

glucose –> sorbitol

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15
Q

sorbitol dehydrogenase

A

sorbitol –> fructose

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16
Q

When is polyol pathway active?

A

at high glucose levels

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17
Q

What tissues express high levels of sorbitol dehydrogenase?

A

liver, ovaries, and seminal vesicles

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18
Q

increased polyol pathway causes _

A

osmotic and oxidative stress

19
Q

Why does polyol pathway increase oxidative stress?

A

aldose reductase requires the conversion of NADPH into NADP+, depleting NADPH in the cell –> NADPH can not form glutathione which results in increased oxidative stress

20
Q

increased sorbitol causes _

A

osmotic stress (cell edema) and decreased ATPase activity (NT delay)

21
Q

symptoms of increased polyol pathway

A

chronic complications including neuropathy, retinopathy, and renal failure

22
Q

tissues with low levels of sorbitol dehydrogenase are susceptible to _

A

high glucose levels leading to diabetic neuropathy and diabetic retinopathy (no sorbitol dehydrogenase)

23
Q

primary lactose intolerance

A

lactase production decreases in adulthood

24
Q

secondary lactose intolerance

A

lactase production decreases after an illness, injury, or small intestine surgery

25
Q

tertiary lactose intolerance

A

congenital lactase deficiency

26
Q

galactose is metabolized by the _

A

Leloir pathway

27
Q

Leloir pathway

A

galactose –> galactose-1-P –> glucose-1-P

28
Q

galactokinase (GALK)

A

galactose –> galactose-1-P

29
Q

galactose 1-P uridylyltransferase (GALT)

A

galactose-1-P –> glucose-1-P; also converts UDP-glucose into UDP-galactose

30
Q

galactosemia

A

results from defect in Leloir pathway

31
Q

GALT deficiency

A

leads to type I galactosemia; most common form

32
Q

GALK deficiency

A

leads to type II galactosemia

33
Q

type II galactosemia treatment

A

galactose and lactose restricted diet, calcium supplements to maintain bone density

34
Q

type II galactosemia symptoms

A

elevated galactose, cataracts due to increased galactitol

35
Q

type I galactosemia symtpoms

A

failure to thrive, liver and renal dysfunction, cataracts, abnormal liver development, and sepsis

36
Q

type I galactosemia treatment

A

low galactose and lactose diet

37
Q

UDP-glucose-4-epimerase (GALE)

A

converts UDP-galactose to UDP-glucose to be made into glycogen

38
Q

GALE deficiency

A

type III galactosemia

39
Q

type III galactosemia symptoms

A

similar to GALT deficiency if severe form

40
Q

type III galactosemia treatment

A

low galactose and lactose treatment

41
Q

mild form of type III galactosemia

A

restricted to blood cells, asymptomatic

42
Q

congenital disorders of glycosylation symptoms

A

low muscle tone, misaligned eyes, poor growth, developmental delay, liver disease, stroke-like episodes, seizures, abnormal bleeding/blood clotting

43
Q

alpha-galactose syndrome

A

caused by an allergic response to glycoproteins (galactose-alpha-1,3-galactose in meat) causing rashes, hives, nausea, vomiting, breathing difficulty, low BP, dizziness, and stomach pain

44
Q

xenobiotics are solubilized by _

A

conjugation to glucuronate for removal by liver