Product info:

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. [provided by RefSeq, Jul 2008]

Alternative names:

PBG-D; PBGD; PORC; UPS

Species reactivity:

Human, Mouse, Rat

Host:

Rabbit

Cellular localisation:

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Isotype:

IgG

Immunogen:

A synthesized peptide derived from human HMBS

Positive control:

Mouse kidney, Mouse heart, HepG2, Rat kidney

AA Sequence:

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Purification:

Affinity purification

Molecular weight:

Calculated MW: 42kDa Observed MW: 42KDa

Form:

Liquid

Applications:

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Dilution:

WB 1:500 - 1:2000

Storage buffer:

Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3.

Storage:

Store at 4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.

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