Anti-PEX6 Antibody (CAB10459)
- SKU:
- CAB10459
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Frequently bought together:
Description
抗体名: | Anti-PEX6 Antibody |
抗体コード: | CAB10459 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB |
反応性: | Human, Mouse, Rat |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 741-980 of human PEX6 (NP_000278.3). |
申し込み: | WB |
推奨希釈: | WB 1:500 - 1:2000 |
反応性: | Human, Mouse, Rat |
ポジティブサンプル: | BT-474, K-562, Mouse liver, Mouse brain |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 741-980 of human PEX6 (NP_000278.3). |
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: | LLHG PPGT GKTL LAKA VATE CSLT FLSV KGPE LINM YVGQ SEEN VREV FARA RAAA PCII FFDE LDSL APSR GRSG DSGG VMDR VVSQ LLAE LDGL HSTQ DVFV IGAT NRPD LLDP ALLR PGRF DKLV FVGA NEDR ASQL RVLS AITR KFKL EPSV SLVN VLDC CPPQ LTGA DLYS LCSD AMTA ALKR RVHD LEEG LEPG SSAL MLTM EDLL QAAA RLQP SVSE QELL RYKR IQRK FAAC |
遺伝子ID: | 5190 |
Uniprot: | Q13608 |
セルラーロケーション: | Cytoplasm, Peroxisome membrane |
計算された分子量: | 77kDa/94kDa/104kDa |
観察された分子量: | 104kDa |
同義語: | PEX6, HMLR2, PAF-2, PAF2, PBD4A, PDB4B, PXAAA1 |
バックグラウンド: | This gene encodes a member of the AAA (ATPases associated with diverse cellular activities) family of ATPases. This member is a predominantly cytoplasmic protein, which plays a direct role in peroxisomal protein import and is required for PTS1 (peroxisomal targeting signal 1, a C-terminal tripeptide of the sequence ser-lys-leu) receptor activity. Mutations in this gene cause peroxisome biogenesis disorders of complementation group 4 and complementation group 6. Several transcript variants encoding different isoforms have been found for this gene. |
UniProt Protein Function: | PEX6: Involved in peroxisome biosynthesis. Required for stability of the PTS1 receptor. Anchored by PEX26 to peroxisome membranes, possibly to form heteromeric AAA ATPase complexes required for the import of proteins into peroxisomes. Defects in PEX6 are the cause of peroxisome biogenesis disorder complementation group 4 (PBD-CG4); also known as PBD-CGC. 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 PEX6 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 AAA ATPase family. |
UniProt Protein Details: | Protein type:Membrane protein, peripheral Chromosomal Location of Human Ortholog: 6p21.1 Cellular Component: cytoplasm; cytosol; peroxisome Molecular Function:ATP binding; ATPase activity; ATPase activity, coupled; protein binding; protein C-terminus binding; protein complex binding Biological Process: peroxisome organization and biogenesis; protein import into peroxisome matrix, translocation; protein stabilization; protein targeting to peroxisome Disease: Heimler Syndrome 2; Peroxisome Biogenesis Disorder 4a (zellweger); Peroxisome Biogenesis Disorder 4b |
NCBI Summary: | This gene encodes a member of the AAA (ATPases associated with diverse cellular activities) family of ATPases. This member is a predominantly cytoplasmic protein, which plays a direct role in peroxisomal protein import and is required for PTS1 (peroxisomal targeting signal 1, a C-terminal tripeptide of the sequence ser-lys-leu) receptor activity. Mutations in this gene cause peroxisome biogenesis disorders of complementation group 4 and complementation group 6. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2015] |
UniProt Code: | Q13608 |
NCBI GenInfo Identifier: | 12644408 |
NCBI Gene ID: | 5190 |
NCBI Accession: | Q13608.2 |
UniProt Secondary Accession: | Q13608,Q5T8W1, Q8WYQ0, Q8WYQ1, Q8WYQ2, Q99476, |
UniProt Related Accession: | Q13608 |
Molecular Weight: | 104kDa |
NCBI Full Name: | Peroxisome assembly factor 2 |
NCBI Synonym Full Names: | peroxisomal biogenesis factor 6 |
NCBI Official Symbol: | PEX6 |
NCBI Official Synonym Symbols: | PAF2; HMLR2; PAF-2; PBD4A; PDB4B; PXAAA1 |
NCBI Protein Information: | peroxisome biogenesis factor 6 |
UniProt Protein Name: | Peroxisome assembly factor 2 |
UniProt Synonym Protein Names: | Peroxin-6; Peroxisomal biogenesis factor 6; Peroxisomal-type ATPase 1 |
Protein Family: | Peroxisomal ATPase |
UniProt Gene Name: | PEX6 |
View AllClose