Anti-PEX16 Antibody (CAB10387)
- SKU:
- CAB10387
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Frequently bought together:
Description
抗体名: | Anti-PEX16 Antibody |
抗体コード: | CAB10387 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB |
反応性: | Human, Mouse |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 132-336 of human PEX16 (NP_004804.1). |
申し込み: | WB |
推奨希釈: | WB 1:500 - 1:2000 |
反応性: | Human, Mouse |
ポジティブサンプル: | A-549, MCF7, HT-1080, SW620, Mouse kidney |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 132-336 of human PEX16 (NP_004804.1). |
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: | KAGL QTSP PIVP LDRE TQAQ PPDG DHSP GNHE QSYV GKRS NRVV RTLQ NTPS LHSR HWGA PQQR EGRQ QQHH EELS ATPT PLGL QETI AEFL YIAR PLLH LLSL GLWG QRSW KPWL LAGV VDVT SLSL LSDR KGLT RRER RELR RRTI LLLY YLLR SPFY DRFS EARI LFLL QLLA DHVP GVGL VTRP LMDY LPTW QKIY FYSW G |
遺伝子ID: | 9409 |
Uniprot: | Q9Y5Y5 |
セルラーロケーション: | Endoplasmic reticulum membrane, Multi-pass membrane protein, Peroxisome membrane |
計算された分子量: | 38kDa/39kDa |
観察された分子量: | 39kDa |
同義語: | PEX16, PBD8A, PBD8B |
バックグラウンド: | The protein encoded by this gene is an integral peroxisomal membrane protein. An inactivating nonsense mutation localized to this gene was observed in a patient with Zellweger syndrome of the complementation group CGD/CG9. Expression of this gene product morphologically and biochemically restores the formation of new peroxisomes, suggesting a role in peroxisome organization and biogenesis. Alternative splicing has been observed for this gene and two variants have been described. |
UniProt Protein Function: | PEX16: Required for peroxisome membrane biogenesis. May play a role in early stages of peroxisome assembly. Can recruit other peroxisomal proteins, such as PEX3 and PMP34, to de novo peroxisomes derived from the endoplasmic reticulum (ER). May function as receptor for PEX3. Defects in PEX16 are the cause of peroxisome biogenesis disorder complementation group 9 (PBD-CG9); also known as PBD-CGD. 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 PEX16 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 peroxin-16 family. 2 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Membrane protein, integral; Membrane protein, multi-pass Chromosomal Location of Human Ortholog: 11p11.2 Cellular Component: endoplasmic reticulum; endoplasmic reticulum membrane; integral to peroxisomal membrane; membrane; peroxisomal membrane; peroxisome Molecular Function:protein binding; protein C-terminus binding Biological Process: ER-dependent peroxisome biogenesis; peroxisome membrane biogenesis; peroxisome organization and biogenesis; protein import into peroxisome matrix; protein import into peroxisome membrane; protein targeting to peroxisome; protein to membrane docking Disease: Peroxisome Biogenesis Disorder 8a (zellweger); Peroxisome Biogenesis Disorder 8b |
NCBI Summary: | The protein encoded by this gene is an integral peroxisomal membrane protein. An inactivating nonsense mutation localized to this gene was observed in a patient with Zellweger syndrome of the complementation group CGD/CG9. Expression of this gene product morphologically and biochemically restores the formation of new peroxisomes, suggesting a role in peroxisome organization and biogenesis. Alternative splicing has been observed for this gene and two variants have been described. [provided by RefSeq, Jul 2008] |
UniProt Code: | Q9Y5Y5 |
NCBI GenInfo Identifier: | 332278135 |
NCBI Gene ID: | 9409 |
NCBI Accession: | Q9Y5Y5.2 |
UniProt Secondary Accession: | Q9Y5Y5,Q9BWB9, |
UniProt Related Accession: | Q9Y5Y5 |
Molecular Weight: | 39,270 Da |
NCBI Full Name: | Peroxisomal membrane protein PEX16 |
NCBI Synonym Full Names: | peroxisomal biogenesis factor 16 |
NCBI Official Symbol: | PEX16 |
NCBI Official Synonym Symbols: | PBD8A; PBD8B |
NCBI Protein Information: | peroxisomal biogenesis factor 16 |
UniProt Protein Name: | Peroxisomal membrane protein PEX16 |
UniProt Synonym Protein Names: | Peroxin-16; Peroxisomal biogenesis factor 16 |
Protein Family: | Peroxisomal membrane protein |
UniProt Gene Name: | PEX16 |
UniProt Entry Name: | PEX16_HUMAN |
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