Thermo Fisher Scientific HMBS Recombinant Rabbit Monoclonal Antibody (ARC0685)
HMBS 단백질을 표적으로 하는 Thermo Fisher Scientific의 재조합 토끼 단클론 항체로, Western blot 및 ELISA에 적합합니다. 인간, 마우스, 랫트 시료에 반응하며, HEK293 세포에서 발현되었습니다. 친화 크로마토그래피로 정제된 고순도 항체입니다.
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Applications and Tested Dilution
| Application | Tested Dilution |
|---|---|
| Western Blot (WB) | 1:500–1:2,000 |
| ELISA | 1 µg/mL |
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human, Mouse, Rat |
| Host / Isotype | Rabbit / IgG |
| Expression System | HEK293 cells |
| Class | Recombinant Monoclonal |
| Type | Antibody |
| Clone | ARC0685 |
| Immunogen | A synthetic peptide corresponding to amino acids 250–350 of human HMBS (P08397) |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | 0.34 mg/mL |
| Purification | Affinity Chromatography |
| Storage Buffer | PBS, pH 7.3, with 50% glycerol, 0.05% BSA |
| Contains | 0.02% sodium azide |
| Storage Conditions | −20°C, Avoid Freeze/Thaw Cycles |
| Shipping Conditions | Ambient (domestic); Wet ice (international) |
| RRID | AB_2849061 |
Product Specific Information
Immunogen sequence:
ERAFLRHLEG GCSVPVAVHT AMKDGQLYLT GGVWSLDGSD SIQETMQATI HVPAQHEDGP EDDPQLVGIT ARNIPRGPQL AAQNLGISLA NLLLSKGAKN I
Target Information
PBGD (porphobilinogen deaminase), also known as hydroxymethylbilane synthase (HMBS), is a cytoplasmic enzyme involved in the heme synthesis pathway. PBGD 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 attacks of neurological dysfunctions with hypertension, tachycardia, peripheral neurologic disturbances, abdominal pain, and elevated levels of aminolevulinic acid and porphobilinogen in urine.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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