Thermo Fisher Scientific HMBS Polyclonal Antibody, MaxPab
HMBS 단백질을 인식하는 Thermo Fisher Scientific의 Rabbit Polyclonal 항체. Western blot 및 Immunocytochemistry에 사용 가능. 친화 크로마토그래피로 정제된 액상 항체로, PBS(pH 7.4)에 보존제 없이 제공. 연구용으로만 사용 가능.
- 카탈로그번호
- H00003145-D01P
- 판매단위
- pk
카탈로그
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Applications and Tested Dilution
| Application | Tested Dilution |
|---|---|
| Western Blot (WB) | 1:1,000 |
| Immunocytochemistry (ICC/IF) | 10 µg/mL |
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human |
| Host / Isotype | Rabbit / IgG |
| Class | Polyclonal |
| Type | Antibody |
| Immunogen | HMBS (NP_000181.2, 1 a.a. – approximately 361 a.a) full-length human protein |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | See Label |
| Purification | Affinity chromatography |
| Storage Buffer | PBS, pH 7.4 |
| Contains | No preservative |
| Storage Conditions | -20°C, Avoid Freeze/Thaw Cycles |
| Shipping Conditions | Ambient (domestic); Wet ice (international) |
Product Specific Information
Sequence of this protein is as follows:
MSGNGNAAAT AEENSPKMRV IRVGTRKSQL ARIQTDSVVA TLKASYPGLQ FEIIAMSTTG DKILDTALSK IGEKSLFTKE LEHALEKNEV DLVVHSLKDL PTVLPPGFTI GAICKRENPH DAVVFHPKFV GKTLETLPEK SVVGTSSLRR AAQLQRKFPH LEFRSIRGNL NTRLRKLDEQ QEFSAIILAT AGLQRMGWHN RVGQILHPEE CMYAVGQGAL GVEVRAKDQD ILDLVGVLHD PETLLRCIAE RAFLRHLEGG CSVPVAVHTA MKDGQLYLTG GVWSLDGSDS IQETMQATIH VPAQHEDGPE DDPQLVGITA RNIPRGPQLA AQNLGISLAN LLLSKGAKNI LDVARQLNDA H
Target Information
PBGD (porphobilinogen deaminase), also designated hydroxymethylbilane synthase, is a cytoplasmic enzyme involved in the heme synthesis pathway.
PBGD belongs to the HMBS (hydroxymethylbilane synthase) family.
Deficiency of PBGD causes errors in pyrrole metabolism, leading to the inherited autosomal disorder acute intermittent porphyria (AIP).
AIP is characterized by acute neurological dysfunctions, hypertension, tachycardia, peripheral neurologic disturbances, 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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