Invitrogen™ HMBS Monoclonal Antibody (OTI3F4)

Mouse Monoclonal Antibody

Supplier:  Invitrogen™ MA526545

Catalog No. PIMA526545

Description

HMBS Monoclonal Antibody for Western Blot, IHC (P)

PBGD (porphobilinogen deaminase), 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.

Specifications

• Antigen: HMBS
• Classification: Monoclonal
• Concentration: 1 mg/mL
• Formulation: PBS with 1% BSA, 50% glycerol and 0.02% sodium azide; pH 7.3
• Gene Accession No.: P08397
• Gene Symbols: Hmbs
• Immunogen: Full length human recombinant protein of HMBS produced in HEK293T cell.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG1

• Applications: Immunohistochemistry (Paraffin), Western Blot
• Clone: OTI3F4
• Conjugate: Unconjugated
• Gene: Hmbs
• Gene Alias: alternative name: porphobilinogen deaminase; HEM3; hemC; HMBS; Hydroxymethylbilane synthase; PBG D; PBGD; PBG-D; PORC; Porphobilinogen deaminase; porphyria, acute; Chester type; pre-uroporphyrinogen synthase; similar to Porphobilinogen deaminase (Hydroxymethylbilane synthase) (HMBS) (Pre-uroporphyrinogen synthase) (PBG-D); T25658; Ups; uroporphyrinogen I synthase; uroporphyrinogen I synthetase; URO-S; Uros1
• Host Species: Mouse
• Purification Method: Affinity Chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 3145
• Content And Storage: -20°C, Avoid Freeze/Thaw Cycles
• Form: Liquid