Datasheet

目录号：A5880

生物描述

特异性	HMBS Rabbit Recombinant mAb detects endogenous levels of total HMBS.
背景	Porphobilinogen deaminase encoded by the HMBS gene is involved in the third step of the heme biosynthetic pathway. It catalyzes the loss of ammonia from the porphobilinogen monomer (deamination) and its subsequent polymerization to a linear tetrapyrrole, which is released as hydroxymethylbilane. The most well-known health issue involving porphobilinogen deaminase is acute intermittent porphyria, an autosomal dominant genetic disorder where insufficient hydroxymethylbilane is produced, leading to a build-up of porphobilinogen in the cytoplasm.

特异性

HMBS Rabbit Recombinant mAb detects endogenous levels of total HMBS.

背景

Porphobilinogen deaminase encoded by the HMBS gene is involved in the third step of the heme biosynthetic pathway. It catalyzes the loss of ammonia from the porphobilinogen monomer (deamination) and its subsequent polymerization to a linear tetrapyrrole, which is released as hydroxymethylbilane. The most well-known health issue involving porphobilinogen deaminase is acute intermittent porphyria, an autosomal dominant genetic disorder where insufficient hydroxymethylbilane is produced, leading to a build-up of porphobilinogen in the cytoplasm.

使用信息

抗体应用

wb,ELISA,ELISA

稀释比例

1:1000

反应性

Human Mouse Rat

MW (kDa)

42kDa

抗体类型

储存液配方

10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/ml BSA, 50% glycerol and less than 0.02% sodium azide.

储存条件
(自收到货起)

Store at –20°C.

文献参考

[1] Grandchamp B, et al. Nucleic Acids Res. 1989, 17(16):6637-49.
[2] Astrin KH, et al. Hum Mutat. 1994, 4(4):243-52.

Application Data

Validated by Selleck