H-HoPro-OH是赖氨酸的分解产物,它能在患有常染色体遗传性疾病的婴儿体液中积累,比如Zellweger综合征,新生儿肾上腺机能障碍。
CAS:3105-95-1
分子式:C6H11NO2
分子量:129.16
纯度:98%
存储:Store at -20°C
Background:
H-HoPro-OH is a breakdown product of lysine, accumulates in body fluids of infants with generalized genetic peroxisomal disorders, such as Zellweger syndrome, neonatal adrenoleukodystrophy.
[1]. Mihalik SJ, et al. Peroxisomal L-pipecolic acid oxidation is deficient in liver from Zellweger syndrome patients. Pediatr Res. 1989 May;25(5):548-52.