Invitrogen™ HMBS Monoclonal Antibody (OTI3F4)

Catalog No.	Quantity
PIMA526545	100 μL

Catalog No. PIMA526545 Supplier Invitrogen™ Supplier No. MA526545

Description

Mouse Monoclonal Antibody

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
• Applications: Immunohistochemistry (Paraffin), Western Blot
• Classification: Monoclonal
• Clone: OTI3F4
• Concentration: 1 mg/mL
• Conjugate: Unconjugated
• Formulation: PBS with 1% BSA, 50% glycerol and 0.02% sodium azide; pH 7.3
• Gene: Hmbs
• Gene Accession No.: P08397
• 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
• Gene Symbols: Hmbs
• Host Species: Mouse
• Immunogen: Full length human recombinant protein of HMBS produced in HEK293T cell.
• Purification Method: Affinity Chromatography
• Quantity: 100 μL
• Regulatory Status: RUO
• Primary or Secondary: Primary
• Gene ID (Entrez): 3145
• Target Species: Human
• Content And Storage: -20°C, Avoid Freeze/Thaw Cycles
• Product Type: Antibody
• Form: Liquid
• Isotype: IgG1