Anti-ZMPSTE24 Antibody (CAB18593)
- SKU:
- CAB18593
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
Frequently bought together:
Description
| 抗体名: | Anti-ZMPSTE24 Antibody |
| 抗体コード: | CAB18593 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB IHC |
| 反応性: | Human, Mouse, Rat |
| 宿主種: | Rabbit |
| 免疫原: | A synthetic peptide corresponding to a sequence within amino acids 200-300 of human ZMPSTE24 (NP_005848.2). |
| 申し込み: | WB IHC |
| 推奨希釈: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 |
| 反応性: | Human, Mouse, Rat |
| ポジティブサンプル: | SKOV3 |
| 免疫原: | A synthetic peptide corresponding to a sequence within amino acids 200-300 of human ZMPSTE24 (NP_005848.2). |
| 精製方法: | Affinity purification |
| ストレージバッファ: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| アイソタイプ: | IgG |
| 順序: | Email for sequence |
| 遺伝子ID: | 10269 |
| Uniprot: | O75844 |
| セルラーロケーション: | |
| 計算された分子量: | |
| 観察された分子量: | 54kDa |
 | Western blot - ZMPSTE24 Rabbit pAb (CAB18593) |
| 同義語: | ZMPSTE24, FACE-1, FACE1, HGPS, PRO1, STE24, Ste24p |
| バックグラウンド: |
| UniProt Protein Function: | ZMPSTE24: Proteolytically removes the C-terminal three residues of farnesylated proteins. Acts on lamin A/C. Defects in ZMPSTE24 are the cause of mandibuloacral dysplasia with type B lipodystrophy (MADB). Mandibuloacral dysplasia (MAD) is a rare autosomal recessive disorder characterized by mandibular and clavicular hypoplasia, acroosteolysis, delayed closure of the cranial suture, joint contractures, and types A or B patterns of lipodystrophy. Type B lipodystrophy observed in MADB, is characterized by generalized fat loss. Defects in ZMPSTE24 are a cause of lethal tight skin contracture syndrome (LTSCS); also called restrictive dermopathy (RD). Lethal tight skin contracture syndrome is a rare disorder mainly characterized by intrauterine growth retardation, tight and rigid skin with erosions, prominent superficial vasculature and epidermal hyperkeratosis, facial features (small mouth, small pinched nose and micrognathia), sparse/absent eyelashes and eyebrows, mineralization defects of the skull, thin dysplastic clavicles, pulmonary hypoplasia, multiple joint contractures and an early neonatal lethal course. Liveborn children usually die within the first week of life. The overall prevalence of consanguineous cases suggested an autosomal recessive inheritance. Belongs to the peptidase M48A family. |
| UniProt Protein Details: | Protein type:Protease; Membrane protein, multi-pass; EC 3.4.24.84; Membrane protein, integral Chromosomal Location of Human Ortholog: 1p34 Cellular Component: endoplasmic reticulum membrane; integral to membrane; membrane; nuclear inner membrane Molecular Function:metal ion binding; metalloendopeptidase activity; metalloexopeptidase activity Biological Process: nuclear membrane organization and biogenesis; prenylated protein catabolic process; proteolysis Disease: Mandibuloacral Dysplasia With Type B Lipodystrophy; Restrictive Dermopathy, Lethal |
| NCBI Summary: | This gene encodes a member of the peptidase M48A family. The encoded protein is a zinc metalloproteinase involved in the two step post-translational proteolytic cleavage of carboxy terminal residues of farnesylated prelamin A to form mature lamin A. Mutations in this gene have been associated with mandibuloacral dysplasia and restrictive dermopathy. [provided by RefSeq, Jul 2008] |
| UniProt Code: | O75844 |
| NCBI GenInfo Identifier: | 18379366 |
| NCBI Gene ID: | 10269 |
| NCBI Accession: | NP_005848.2 |
| UniProt Secondary Accession: | O75844,Q8NDZ8, Q9UBQ2, B3KQI7, D3DPU7, |
| UniProt Related Accession: | O75844 |
| Molecular Weight: | 54,813 Da |
| NCBI Full Name: | CAAX prenyl protease 1 homolog |
| NCBI Synonym Full Names: | zinc metallopeptidase STE24 |
| NCBI Official Symbol: | ZMPSTE24 |
| NCBI Official Synonym Symbols: | HGPS; PRO1; FACE1; STE24; FACE-1; Ste24p |
| NCBI Protein Information: | CAAX prenyl protease 1 homolog |
| UniProt Protein Name: | CAAX prenyl protease 1 homolog |
| UniProt Synonym Protein Names: | Farnesylated proteins-converting enzyme 1; FACE-1; Prenyl protein-specific endoprotease 1; Zinc metalloproteinase Ste24 homolog |
| UniProt Gene Name: | ZMPSTE24 |
| UniProt Entry Name: | FACE1_HUMAN |