Test 23- BC Flashcards
Glycogen degradation in skeletal muscle increases several hundred fold after the onset of contraction. This is due to enzyme activation by which substance?
Ca
What couples glycogen degradation to skeletal muscle contraction?
Calcium mediated myophosphorylase activation
Increased Ca in the cytosol activates phosphorylase kinase>
activates muscle phosphorylase
Maple syrup urine disease
defect in branched chain alpha ket acid DH>
inability to break down (LIV) beyond their deaminated alpha keto acid state>
elevated levels lead to neurotoxicity
Dystonia (sustained muscle contractions), poor feeding, maple syrup scent of urine
MSUD
Tx for MSUD
restrict branched chain A
Krebs cycle mneumonic
Citrate is = isocitrate krebs= alpha keto starting= succinyl coa substrate= succinate for making oxaloacteate
Branched chain AA
leucine
isoleucine
valine
Restrict in pts w/ PKU
phenylalanine
Defective breakdown of tyrosine
alkaptonuria
What is the major AA responsible for transferring N to the liver for disposal?
alanine
What happens to amino groups during protein catabolism?
Transferred to alpha ketoglutarate to form GLUTAMATE
What happens to glutamate generated from catabolism of proteins?
Glutamate is processed in the liver to form urea which is disposed of
malate, citrate, OAA
intermediates of TCA cycle
L- citrulline
UREA cycle
AA produced as intermediate in conversion of ornithine to arginosuccinate during urea sycle
What forms calactitol?
excess circulating galactose in galactosemia by Aldose reductase
converts UDP galactose to UDP glucose
UDP galactose 4 epimerase
Beta galctosidase def
leads to accumulation of glycosaminoglycans (keratin sulfate in lysosomes)
def in aldolase B
hereditary frustose inolerance