Amino acid synthesis and degradation and urea cycle :/ Flashcards

1
Q

How are amino acids obtained

A

diet
synthesised by body
produced from normal protein turnover (recycled)

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2
Q

what happens if aa are not required

A

degredated

can be used as a source of energy

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3
Q

can amino acids be stored or not

A

cannot be stored

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4
Q

List some essential aa from the diet

A
histidine
isoleucine
leucine
lysine
methaionaine
phenylalanine
threonine
typtophan
valine
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5
Q

non essential aa examples

A
alanine
aspargine
aspartate
glutamate
serine
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6
Q

conditionally essential aa examples

A
arginine
cysteine
glutamine
glycine
proline
tyrosine
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7
Q

what is cystinuria

A

defective carrier system
- leads to aa in urnine
crystals of cysteine form kidney stones

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8
Q

what happens when nitrogen is consumed in the diet

A

usually consumed in a form of protien
- hydrolysed to aa by proteolytic enzymes
aa AT into epithelial cells and enter blood

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9
Q

what are the proteolytic enzymes and where are they found

A

pepsin in stomach

trypsin in pancreas

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10
Q

what is required to make aa

A

carbon

nitrogen

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11
Q

what can aa be made from

A

glucose and nitrogen source) aa or ammonia)

can be made from intermediates of glycolysis and the TCA cyecle

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12
Q

what amino acids can be made from aa

A

non essential amino acids can be made from essential

- donate molecule

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13
Q

why would aa be needed to degradate

A

excess in diet

some no longer needed

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14
Q

types of aa

A

Glucogenic aa

ketogenic aa

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15
Q

degredation of glycogenic aa

A

carbons converted to glucose

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16
Q

degradation of ketogenic

A

converted to acetylene Ca or acetoacetate(ketone bodies)

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17
Q

what does the amino acid split into

A

carbon skeleton

amino acid nitrogen

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18
Q

how is nitrogen removed from aa

A

transamination

- amino group from one aa transferred to another

19
Q

removal of aa nitrogen as ammonia

A

glutamate can collect nitrogen from other aa
converted to ammonia by glutamate dehydrogenase in the liver
aa enters urea cycle

20
Q

what is removal aa nitrogen as ammonia alllostericaly alters by

A

allosteric regualtion by :

  • ATP and GTP (inhibits)
  • ADP and Gdp activates
21
Q

lysosome

A

protease filled vesicles

22
Q

ubituitin

A

small protiens that target proteins for degredation

23
Q

proteasome

A

protease complex
proitne is unfolded and degraded
- easier for the protein to be degredated by proteases

24
Q

what form of nitrogen is not usable in biological systems

25
nitrogen that is usabel
NH3
26
nitrogen that is toxic
NH4+
27
nitrogen balance
nitrogen ingested (proteins)= nitrogen excreted
28
children and preganen woman nitrogen balance
+
29
disease / starvation nitrogen balance
-
30
nitrogen cycle
``` 5 main steps 1st 2 in mitochondrion other 3 in cytosol nitrogen enters as NH4+ and aspartate ornithine initiates and is regenerated ```
31
control of the urea cyle
feed forward regulation allosteric activate of enzymes high protein diet or fasting induces urea cycle ensymes
32
feed forward urea control
high rate of ammonia production higher rate of urea
33
what enzymes allosterically control the urea cycle
arginine stimulates carbonyl phosphate synthase§
34
what is the glucose alanine cycle
cycling of nutrients between muscles and liver
35
what does the glucose alanine cycle dependon
physiological state i.e. fed or fastine
36
what occurs on fasting
muscle protein broken down to aa transaminate to form alanine transported to liver nitrogen enters urea cycle and pyruvate used to make energy (glucose)
37
what are Ketone bodies
combination of acetoacetate and beta hydroxybutyrate
38
what happens to Ketone bodies when glucose is low
reconverted to acetylene coA | enter TCA cycle for entry
39
what can acetoacetate break down to
acetone spontaneously
40
what is a diagnostic tool for ketonic states
fruity breath
41
Phenylketonuria
mutation in phenylalanine hydroxylate leads to metal retardation affects nervous tissue
42
urea cycle disroder
accumulation of ammonia | toxic to NS
43
inborn errors of aa metabolism
deficient enzymes in aa metabolism | leads to accumulation of harmful producs
44
what are some inborn errors in aa metabolism examples
phenylketonura | urea cycle disorders