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HMBS Antibody - middle region : FITC (ARP54452_P050-FITC)

Product Specifications

Gene Name

Hydroxymethylbilane synthase

Gene Aliases

UPS, PBGD, PORC, PBG-D

Gene ID

3145

Swiss Prot

P08397

Accession Number

NP_000181

Host

Rabbit

Reactivity

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

Immunogen

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

Target

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.

Partner Proteins

RANBP9; APP; UBC

Clonality

Polyclonal

Conjugation

FITC: Fluorescein Isothiocyanate

Type

Polyclonal Antibody

Applications

WB

Purification

Affinity Purified

Concentration

0.5 mg/ml

Homology

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

Format

Liquid. Purified antibody supplied in 1x PBS buffer.

Reconstitution

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.

Molecular Weight

40kDa

References & Citations

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

Shipping Conditions

Wet Ice

Protein Length

361

NCBI Gene Symbol

HMBS

NCBI GB Accession Number

HMBS

Host or Source

Rabbit

Protein Name

Porphobilinogen deaminase

Nucleotide Accession Number

NM_000190

Peptide Sequence

SSLRRAAQLQRKFPHLEFRSIRGNLNTRLRKLDEQQEFSAIILATAGLQR

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