Thermo Fisher Scientific HMBS Monoclonal Antibody (OTI3F4)
HMBS 단백질을 인식하는 Thermo Fisher Scientific의 단클론 항체. Western blot 및 IHC(P) 검증 완료. 인간 시료에 반응하며, 마우스 IgG1 아이소타입. 액상 형태로 제공되며, -20°C에서 보관 권장.
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Applications and Tested Dilutions
| Application | Tested Dilution |
|---|---|
| Western Blot (WB) | 1:2,000 |
| Immunohistochemistry (Paraffin) (IHC (P)) | 1:150 |
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human |
| Host / Isotype | Mouse / IgG1 |
| Class | Monoclonal |
| Type | Antibody |
| Clone | OTI3F4 |
| Immunogen | Full length human recombinant protein of HMBS produced in HEK293T cells |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | 1 mg/mL |
| Purification | Affinity Chromatography |
| Storage Buffer | PBS, pH 7.3, with 1% BSA, 50% glycerol |
| Contains | 0.02% sodium azide |
| Storage Conditions | -20°C, Avoid Freeze/Thaw Cycles |
| Shipping Conditions | Ambient (domestic); Wet ice (international) |
| RRID | AB_2724460 |
Target Information
PBGD (porphobilinogen deaminase), also known as hydroxymethylbilane synthase (HMBS), is a cytoplasmic enzyme involved in the heme biosynthesis pathway. It belongs to the HMBS family. Deficiency of PBGD results in errors in pyrrole metabolism, leading to an inherited autosomal disorder called acute intermittent porphyria (AIP).
AIP is characterized by acute neurological dysfunctions such as hypertension, tachycardia, peripheral neuropathy, abdominal pain, and elevated urinary levels of aminolevulinic acid and porphobilinogen.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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