Thermo Fisher Scientific HMBS Polyclonal Antibody
HMBS 단백질을 인식하는 Thermo Fisher Scientific의 Rabbit Polyclonal Antibody. Western blot, IHC, ICC/IF 등 다양한 응용에 사용 가능. 고순도 Affinity chromatography 정제, PBS/glycerol buffer 보존. 연구용으로만 사용.
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Applications
| Application | Tested Dilution |
|---|---|
| Western Blot (WB) | 1:500–1:2,000 |
| Immunohistochemistry (Paraffin) (IHC (P)) | 1:50–1:200 |
| Immunocytochemistry (ICC/IF) | 1:100–1:500 |
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human, Mouse, Rat |
| Host / Isotype | Rabbit / IgG |
| Class | Polyclonal |
| Type | Antibody |
| Immunogen | A synthesized peptide derived from human HMBS (Accession P08397), corresponding to amino acid residues K140–T190 |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | 1 mg/mL |
| Purification | Affinity chromatography |
| Storage Buffer | PBS, pH 7.4, with 50% glycerol |
| Contains | 0.02% sodium azide |
| Storage Conditions | -20°C |
| Shipping Conditions | Wet ice |
| RRID | AB_2851884 |
Product Specific Information
Antibody detects endogenous levels of total HMBS.
Target Information
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, leading 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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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