cystinosin
DESCRIPTION
cystinosin. cytosole. Cysteine –cysteamine. cystine. cysteamine. cysteine. NH 2 COOH CH CH 2 S S CH 2 CH NH 2 COOH. NH 2 COOH CH CH 2 S S CH 2 - PowerPoint PPT PresentationTRANSCRIPT
cystinosin
NH2 COOH
CH
CH2
S
S
CH2
CH
NH2 COOH
+HS
CH2
CH2
NH2
SH
CH2
CH
NH2 COOH
NH2 COOH
CH
CH2
S
S
CH2
CH2
NH2
+
cystine cysteamine cysteineCysteine –cysteamine
lysine transporter
cytosole
lysosome
Cysteine transporter
0,0
0,2
0,4
0,6
0,8
1,0
cyst
ine
nmol
/mg
prot
ein
9 hours after
CystagonR dose
6 hours after
CystagonR dose
p < 0.05