Protein and AMino Acid Metabolism IV Flashcards
actual or function vit B12 defiency results in what
progressive neurlogical impariemnt
there is a strong correlation between high levels of homocyseine and the development of what disease - most likely as a marker
atheroslerosis
what coenzymes/vitamins does the homocysteine metabolism require
vit B6, B12, foalte
waht does valine catabolism producee
propionyl CoA
what does isoleucine catabolism produce
propionyl CoA adn acetyl CoA
what does catabolism of leucine prodcue
acetyl-CoA and acetoacetate
what does catabolism of BCAA outside of liver require
branched chain amino transferases, BCKDH
maple syrup urine disease is caused by waht
defect in BCKDH compelx
symptoms of maple syrup urine diseas
poor feeding, vomiting, slow/irregular breathing, ketoacidosis
how do you treat maple syrup urinse disease
reduced BCAAs in diet
what population is maple syrup urine disease more common in
old order mennonite
what casues tyrosinemia II
tyrosine amino acid transferase defect
what casues alcaptonuria
homogentisate oxidase defect
what casues tyrosinemia I
fumarylacetoacetate hydrolase defeect
symptoms of tyrosinemia II
keratitis
photophobia
painful skin lesion
intellectual disbiality
symptoms of alkaptonuria
black urine
symptoms of tyrosinemia I
liver, kidney, neurologic disfunction
srouces of ammonium ions
transfer of alpha mino groups to glutamate durign aminotransferase reaction
deamination of serine, cysteine, histidien and theronein
action of glutaminase and asparginase
what happens to most of the ammonium inos genreated during catabolism
incorporating into urea by liver
during normal metabolism the liver mitochondria genereate what
HCO3, CO2, NH4, ATP
what is the rate determing step of urea synthesis
formation of carbomoyl phosphate
what does carbamoyl phosphate synthetase I require
N acetylglutamate
how is the urea cycle linked to the TCA cycle
release of fumarte goes to TCA cycle which releases oxaloacetate which converts into aspartate which goes into urea cycle
when is the interstinal-renal axis established
postnatally
what is the only organ that can perform the entire urea cycle
liver
when is arginine essential in adults
conditions that impact function of small intestine or kidney
citrullinemia results from waht
defect in arginnosuccinate synthase
defect in what causes argininosuccinic aciduria
argininosuccinate lyase
what causes hyperargininemia
defect in arginase
how do you treat newborns with early cycle defects
protein restriction
hemodialysis
phenylbutyrate and benzoate
symptoms of hyeprammonenia
lethargy, stupar, vomiting, convulsions
what important thing is happening in astrocytes
main site of brain where glutamate is converted to glutamine
what happens after 10-12 horus of fasting
hepatic gluconeogensis required to supply glucose
the action of BCAT on BCAAs is a major source of what
nitrogen
