HMBS Antilichaam - Middengebied: FITC (ARP54452 P050-FITC)

Hydroxymethylbilane synthase

UPS, PBGD, PORC, PBG-D

3145

P08397

NP_000181

Human, Mouse, Rat, Cow, Dog, Goat, Guinea Pig, Horse, Rabbit, Sheep, Zebrafish

The immunogen is a synthetic peptide directed towards the middle region of human HMBS

HMBS is a member of the hydroxymethylbilane synthase superfamily. It is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria.This gene encodes a member of the hydroxymethylbilane synthase superfamily. The encoded protein is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria. Alternatively spliced transcript variants encoding different isoforms have been described.

RANBP9; APP; UBC

FITC: Fluorescein Isothiocyanate

Polyclonal Antibody

WB

Affinity Purified

0.5 mg/ml

Cow: 93%; Dog: 93%; Goat: 93%; Guinea Pig: 100%; Horse: 93%; Human: 100%; Mouse: 93%; Rabbit: 100%; Rat: 100%; Sheep: 93%; Zebrafish: 91%

Liquid. Purified antibody supplied in 1x PBS buffer.

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

40kDa

Kuo, H.C., J. Neurol. Sci. 260 (1-2), 231-235 (2007)

Wet Ice

361

HMBS

HMBS

Rabbit

Porphobilinogen deaminase

NM_000190

SSLRRAAQLQRKFPHLEFRSIRGNLNTRLRKLDEQQEFSAIILATAGLQR