Anti-PEX12 Antibody (CAB16062)
- SKU:
- CAB16062
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
| 抗体名: | Anti-PEX12 Antibody |
| 抗体コード: | CAB16062 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB |
| 反応性: | Human |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 290-359 of human PEX12 (NP_000277.1). |
| 申し込み: | WB |
| 推奨希釈: | WB 1:500 - 1:2000 |
| 反応性: | Human |
| ポジティブサンプル: | U-87MG, MCF7 |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 290-359 of human PEX12 (NP_000277.1). |
| 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: | YNSD SPLL PKMK TVCP LCRK TRVN DTVL ATSG YVFC YRCV FHYV RSHQ ACPI TGYP TEVQ HLIK LYSP EN |
| 遺伝子ID: | 5193 |
| Uniprot: | O00623 |
| セルラーロケーション: | Multi-pass membrane protein, Peroxisome membrane |
| 計算された分子量: | 40kDa |
| 観察された分子量: | 41kDa |
| 同義語: | PEX12, PAF-3, PBD3A |
| バックグラウンド: | This gene belongs to the peroxin-12 family. Peroxins (PEXs) are proteins that are essential for the assembly of functional peroxisomes. The peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous autosomal recessive, lethal diseases characterized by multiple defects in peroxisome function. The peroxisomal biogenesis disorders are a heterogeneous group with at least 14 complementation groups and with more than 1 phenotype being observed in cases falling into particular complementation groups. Although the clinical features of PBD patients vary, cells from all PBD patients exhibit a defect in the import of one or more classes of peroxisomal matrix proteins into the organelle. Defects in this gene are a cause of Zellweger syndrome (ZWS). |
| UniProt Protein Function: | PEX12: Required for protein import into peroxisomes. Defects in PEX12 are the cause of peroxisome biogenesis disorder complementation group 3 (PBD-CG3). PBD refers to a group of peroxisomal disorders arising from a failure of protein import into the peroxisomal membrane or matrix. The PBD group is comprised of four disorders: Zellweger syndrome (ZWS), neonatal adrenoleukodystrophy (NALD), infantile Refsum disease (IRD), and classical rhizomelic chondrodysplasia punctata (RCDP). ZWS, NALD and IRD are distinct from RCDP and constitute a clinical continuum of overlapping phenotypes known as the Zellweger spectrum. The PBD group is genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies. Defects in PEX12 are a cause of Zellweger syndrome (ZWS). ZWS is a fatal peroxisome biogenesis disorder characterized by dysmorphic facial features, hepatomegaly, ocular abnormalities, renal cysts, hearing impairment, profound psychomotor retardation, severe hypotonia and neonatal seizures. Death occurs within the first year of life. Belongs to the pex2/pex10/pex12 family. |
| UniProt Protein Details: | Protein type:Membrane protein, multi-pass; Membrane protein, integral; Ubiquitin conjugating system Chromosomal Location of Human Ortholog: 17q12 Cellular Component: integral to peroxisomal membrane; peroxisomal membrane; peroxisome Molecular Function:protein binding; protein C-terminus binding; ubiquitin-protein ligase activity; zinc ion binding Biological Process: peroxisome organization and biogenesis; protein import into peroxisome matrix; protein monoubiquitination Disease: Peroxisome Biogenesis Disorder 3a (zellweger); Peroxisome Biogenesis Disorder 3b |
| NCBI Summary: | This gene belongs to the peroxin-12 family. Peroxins (PEXs) are proteins that are essential for the assembly of functional peroxisomes. The peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous autosomal recessive, lethal diseases characterized by multiple defects in peroxisome function. The peroxisomal biogenesis disorders are a heterogeneous group with at least 14 complementation groups and with more than 1 phenotype being observed in cases falling into particular complementation groups. Although the clinical features of PBD patients vary, cells from all PBD patients exhibit a defect in the import of one or more classes of peroxisomal matrix proteins into the organelle. Defects in this gene are a cause of Zellweger syndrome (ZWS). [provided by RefSeq, Oct 2008] |
| UniProt Code: | O00623 |
| NCBI GenInfo Identifier: | 3024371 |
| NCBI Gene ID: | 5193 |
| NCBI Accession: | O00623.1 |
| UniProt Secondary Accession: | O00623,B2R6M2, |
| UniProt Related Accession: | O00623 |
| Molecular Weight: | 40,797 Da |
| NCBI Full Name: | Peroxisome assembly protein 12 |
| NCBI Synonym Full Names: | peroxisomal biogenesis factor 12 |
| NCBI Official Symbol: | PEX12 |
| NCBI Official Synonym Symbols: | PAF-3; PBD3A |
| NCBI Protein Information: | peroxisome assembly protein 12 |
| UniProt Protein Name: | Peroxisome assembly protein 12 |
| UniProt Synonym Protein Names: | Peroxin-12; Peroxisome assembly factor 3; PAF-3 |
| Protein Family: | Peroxisome assembly protein |
| UniProt Gene Name: | PEX12 |
| UniProt Entry Name: | PEX12_HUMAN |
 | Western blot analysis of extracts of various cell lines, using PEX12 antibody (CAB16062) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 3s. |
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