HMBS antibody

Catalog No. ABIN1386078

Product Details anti-HMBS Antibody

Target: HMBS (Hydroxymethylbilane Synthase (HMBS))

Reactivity: Human, Mouse, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This HMBS antibody is un-conjugated

Application: Western Blotting (WB), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Cross-Reactivity: Human, Mouse, Rat

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human HMBS/PBGD

Isotype: IgG

Application Details

Application Notes: WB 1:300-5000
IHC-P 1:200-400
IF(IHC-P) 1:50-200

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: 4 °C,-20 °C

Storage Comment: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Expiry Date: 12 months

Target Details for HMBS

Target: HMBS (Hydroxymethylbilane Synthase (HMBS))

Alternative Name: HMBS (HMBS Products)

Synonyms: PBG-D antibody, PBGD antibody, PORC antibody, UPS antibody, URO-S antibody, hemC antibody, pbg-d antibody, pbgd antibody, ups antibody, SPAC806.01 antibody, DDBDRAFT_0186148 antibody, DDBDRAFT_0231417 antibody, DDB_0186148 antibody, DDB_0231417 antibody, Hmbs antibody, NV50236 antibody, T25658 antibody, Ups antibody, Uros1 antibody, hmbs antibody, zgc:64128 antibody, hmbsl antibody, id:ibd5004 antibody, im:7140060 antibody, zgc:110690 antibody, hydroxymethylbilane synthase antibody, hydroxymethylbilane synthase S homeolog antibody, hydroxymethylbilane synthase L homeolog antibody, hydroxymethylbilane synthase (predicted) antibody, Hydroxymethylbilane synthase antibody, hydroxymethylbilane synthase a antibody, hydroxymethylbilane synthase, b antibody, HMBS antibody, Hmbs antibody, hmbs antibody, hmbs.2.S antibody, hmbs.L antibody, CNC02250 antibody, hem3 antibody, hemC antibody, Trad_0335 antibody, Plabr_2100 antibody, Sgly_3113 antibody, SMLT_RS19660 antibody, PMI_RS16580 antibody, hmbsa antibody, hmbsb antibody

Background:

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.

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.

Gene ID: 3145