CC GNE Flashcards
ultimate end product of protein
AMINO ACID
Basic structure of an amino acid
amino group
R group
carboxyl group
hydrogen
carbon- central part
enzymes that are responsible for digestion of protein
pepsin
trypsin
What is DEAMINATIOM
enzyme for deaminatiom
where do deamination happens
Deamination is the removal of amino group
DEAMINASE
kidneys and liver
WHAT IS TRANSAMINATION
removal of amino group but will be transferred to a keto acid
these are amino acids that can be converted into glucose
glucogenic amino acid
examples of glucogenic AA and its deaminated form
alanine- pyrivate
arginine- a ketoglutarate
aspartate- oxaloacetate
amino acids that generates ketone bodies
ketogenic AA
what do ketone bodies degrade
acetyl-coA
example of ketogenic AA
leucine
lysine
differentiate essential AA from non-essential AA
essential- come from food
non-essential- naturally made by body
NAME THE ESSENTIAL AMINO ACIDS
PVTTIMHALL
fasting for AA
6-8 hrs
best anticoag for plasma (AA)
Heparin
AA- if analysis cannot be done store in
-20- x- 40
Urine is used to (quantify/qualify) level of amino acids?
sample should be collected for
preservative
quantify
24 hrs
thymol
what happens when WBCs are elevated
affects AA determination (aspartic, glitamic)
absence of phenylalanine hydroxylase
PHENYLKETONURIA
what do PAH convert
phenylalanine to tyrosine
a drug used to manage PKI
Kuvan
semi quantitaive test to determine level of phenylalanine
Guthrie
used to quantify conc of phenylalanine
HPLC
inhibits break down of tyrosine
tyrosinemia
metabolite of tyrosine
succinylacetone
Patient has microcephaly and has musty odor urine
Phenylketonuria
Most severe.
Urine is cabbage like
Low levels of Fumarylacetoacetate Hydrolase
Type 1 Tyrosinemia
deficiency in tyrosine aminotransferase
Type 2 Tyrosinemia
Most rare.
Mainly found in liver
Type 3 Tyrosinemia
Urine turns brownish black when exposed to air
Alkaptonuria