Proteins Skildum Flashcards

1
Q

Protein malnutrition is called?

A

Kwashiorkor

*Adequate calories but deficient specifically in protein

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2
Q

Pellega

A

Niacin def.

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3
Q

The protein whose loss causes cystic fibrosis normally functions as a…?

A

Chloride channel

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4
Q

The oxidized form of cysteine is called…?

A

Cystine

making a disulfide bond

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5
Q

The apoproteins ApoB100 and Apo B48 are encoded on the same gene. The different apoproteins are produced through…?

A

RNA editing

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6
Q

How does protein enter the blood?

A

As individual amino acids

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7
Q

What denatures proteins?

A

The low pH of the stomach

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8
Q

What cleaves a zymogen to activate it?

A

Peptidase

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9
Q

Why is secretion of proteases deficient in patients with CF?

A

Because of blockage of the pancreatic duct! This duct carries pancreatic enzymes into the lumen of the small intestine

*As a result they can be susceptible to protein malnutrition

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10
Q

How are amino acids transported across intestinal epithelial cells to the blood?

A

Secondary active transport using sodium

**Similar to SLGT in the kidney!

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11
Q

What transporter, when mutated, results in Hartnup Disease?

A

B0 (SLC6A9)

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12
Q

What is Hartnup Disease?

A

Tryptophan cannot be absorbed or reabsorbed by the sodium-dependent transporter so therefore cannot be used to synthesize niacin. This results in the sx of pellagra due to the niacin deficiency!

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13
Q

What is niacin used to make?

A

NADH and NADPH

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14
Q

What transporter, when mutated, causes cystinuria?

Why??

A

B0+ (SLC7A9)

This transporter brings in basic amino acids, without them…cystine congretates.

**Cystine congregates in proximal tubule causing kidney stone

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15
Q

Only enzyme that can break the bond between Glutamate and cysteine in glutathione?

A

Gamma-glutamyltranspeptidase

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16
Q

What is the outcome of gamma-glutamyltransptptidase?

A

Glutamate bonded to an amino acid along with a cysteinylglycine molecule!

*Transports an amino acid across cellular membranes but isn’t a major contributor to the transport of dietary amino acids but it IS important in glutathione synthesis.

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17
Q

What are the sources of the intracellular amino acid pool (3)?

A

Extracellular amino acids

Protein degradation

de novo synthesis from glycolysis or TCA cycle intermediates!

18
Q

What type of hormone is insulin: catabolic or anabolic?

A

Anabolic

19
Q

What complex activates protein synthesis and inhibits autophagy?

A

mTORC1

20
Q

When activated, what inhibits protein synthesis and promotes autophagy?

A

AMPK

21
Q

What is autophagy?

A

“self-eating”…The large scale version of turning proteins into amino acids

22
Q

What is the key vitamin cofactor in protein metabolism?

What is it key for?

A

PLP-Pyridoxal phosphate

Transaminations, deaminations, carbon chain transfers

23
Q

What is tetrahydrofolate (FH4) key for?

A

One carbon transfers

24
Q

What is tetrahydrobiopterin (BH4) key for?

A

Ring hydroxylations

Ex. Phenylalanine –> Tyrosine requires this!

25
Q

Without PLP, what can manifest?

A

seizures, diarrhea, anemia, EEG abnormalities

26
Q

What can manifest without FH4?

A

megaloblastic anemia

27
Q

What can manifest without BH4?

A

seizures, developmental delays

28
Q

What drug activates AMPK?

A

Metformin

29
Q

What activates trypsinogen?

A

Enteropeptidase

30
Q

What activates pepsinogen?

A

H+

31
Q

Why is secretion of proteases deficient in CF?

A

Blockage of pancreatic duct

32
Q

What lipoprotein activates EC lipase in capillary beds?

A

ApoCII **Allows chylomicron/VLDL to do its job of delivering fatty acids to tissues

33
Q

What does secretin do to the pancreatic duct?

A

Causes contraction of it so contents squirt into the small intestine

34
Q

Lipase cuts at what positions of TAG?

A

1 and 3

35
Q

PLA-2 cuts at what position of TAG?

A

2

36
Q

What length of fatty acids are repackaged into chylomicrons?

A

Only long chain!

37
Q

Apolipoprotein of chylomicrons

A

ApoB48

38
Q

Apolipoprotein of VLDL

A

ApoB100

39
Q

Whats bigger, chylomicron of VLDL?

A

chylomicron (has more triglyceride) but is less dense

40
Q

Familial hypercholesterolemia

A

Mutation in LDL R so LDL can’t be taken up by liver and other tissues

41
Q

Function of ApoE?

A

Mediates remnant uptake!