Metabolism Antibodies 1

Anti-PEX6 Antibody (CAB10459)

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SKU:
CAB10459
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Metabolism

Description

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Antibody Name:Anti-PEX6 Antibody
Antibody SKU:CAB10459
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 741-980 of human PEX6 (NP_000278.3).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse, Rat
Positive Samples:BT-474, K-562, Mouse liver, Mouse brain
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 741-980 of human PEX6 (NP_000278.3).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence: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
Gene ID:5190
Uniprot:Q13608
Cellular Location:Cytoplasm, Peroxisome membrane
Calculated MW:77kDa/94kDa/104kDa
Observed MW:104kDa
Synonyms:PEX6, HMLR2, PAF-2, PAF2, PBD4A, PDB4B, PXAAA1
Background: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  
Anti-PEX6 Antibody (CAB10459)Western blot analysis of extracts of various cell lines, using PEX6 antibody (CAB10459) 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: 5s.
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