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HMBS Polyclonal Antibody, Cy3 Conjugated

Product Specifications

Background

PBGD, also designated hydroxymethylbilane synthase, is a cytoplasmic enzyme found in the heme synthesis pathway. PBGD belongs to the HMBS (hydroxymethylbilane synthase) family. Deficiency of PBGD causes errors in pyrrole metabolism, which in turn leads to an inherited autosomal disorder called acute intermittent porphyria (AIP) . AIP is characterized by acute attacks of neurological dysfunctions with hypertension, tachycardia, peripheral neurologic disturbances, abdominal pain and excessive amounts of aminolevulinic acid and porphobilinogen in the urine.

Synonyms

HEM3_HUMAN; HMBS; Hydroxymethylbilane synthase; PBG D; PBG-D; PBGD; Porphobilinogen deaminase; Pre uroporphyrinogen synthase; Pre-uroporphyrinogen synthase; UPS; Uroporphyrinogen I synthase; Uroporphyrinogen I synthetase.

Gene ID

3145

Host

Rabbit

Cross Reactivity

Human, Mouse, Rat

Immunogen

21-120/361

Target

HMBS

Clonality

Polyclonal

Isotype

IgG

Conjugation

Cy3

Source

KLH conjugated synthetic peptide derived from human HMBS/PBGD

Applications

IF

Purification

Purified by Protein A.

Excitation Emission

512,550nm/570,615nm

Concentration

1µg/µl

Dilution

IF (IF=1:50-200)

Buffer

Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Modification

Unmodified

Storage Conditions

Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Gene ID URL

3145

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