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Anti-HMBS Antibody Picoband® Fluoro647 Conjugated

Product Specifications

Background

Porphobilinogen deaminase (hydroxymethylbilane synthase, or uroporphyrinogen I synthase) is an enzyme that in humans is encoded by the HMBS gene. 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.

Synonyms

Porphobilinogen deaminase; PBG-D; Hydroxymethylbilane synthase; HMBS; Pre-uroporphyrinogen synthase; HMBS; PBGD; UPS

Gene Name

HMBS

Gene ID

3145

UniProt

P08397

Host

Rabbit

Reactivity

Human, Mouse, Rat

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

E. coli-derived human HMBS recombinant protein (Position: N6-H361) .

Clonality

Polyclonal

Tissue Specificity

Isoform 1 is ubiquitously expressed. Isoform 2 is found only in erythroid cells.

Applications

Flow Cytometry

Field of Research

Cell Biology, Cofactors, Vitamins/Minerals, Metabolism, Pathways and Processes, Signal Transduction, Vitamins/Minerals

Purification

Immunogen affinity purified.

Form

Liquid

Function

Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.

References & Citations

1. Lannfelt L, Wetterberg L, Lilius L, Thunell S, Jörnvall H, Pavlu B, Wielburski A, Gellerfors P (November 1989) . Porphobilinogen deaminase in human erythrocytes: purification of two forms with apparent molecular weights of 40 kDa and 42 kDa. Scand. J. Clin. Lab. Invest. 49 (7) : 677–84. 2. Louie GV, Brownlie PD, Lambert R, Cooper JB, Blundell TL, Wood SP, Warren MJ, Woodcock SC, Jordan PM (September 1992) . Structure of porphobilinogen deaminase reveals a flexible multidomain polymerase with a single catalytic site. Nature. 359 (6390) : 33–9.

Storage Conditions

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

Calculated Molecular Weight

39 kDa

Specificity

No cross reactivity with other proteins.

Applications Notes

6

Gene Name Synonym

Hydroxymethylbilane synthase

Subcellular Location

Cytoplasm.

Isotype

Rabbit IgG

Contents

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.

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