Metabolism Antibodies 1

Anti-PEX2 Antibody (CAB15308)

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SKU:
CAB15308
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-PEX2 Antibody
Antibody SKU:CAB15308
Antibody Size:20uL, 50uL, 100uL
Application:WB IHC
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-130 of human PEX2 (NP_001165558.1).
Application:WB IHC
Recommended Dilution:WB 1:200 - 1:2000 IHC 1:50 - 1:200
Reactivity:Human, Mouse, Rat
Positive Samples:LO2, 293T, HeLa, U-251MG, NIH/3T3, Mouse liver, Mouse brain, Rat liver, Rat brain
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-130 of human PEX2 (NP_001165558.1).
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:MASR KENA KSAN RVLR ISQL DALE LNKA LEQL VWSQ FTQC FHGF KPGL LARF EPEV KACL WVFL WRFT IYSK NATV GQSV LNIK YKND FSPN LRYQ PPSK NQKI WYAV CTIG GRWL EERC YDLF RNHH LA
Gene ID:5828
Uniprot:P28328
Cellular Location:Multi-pass membrane protein, Peroxisome membrane
Calculated MW:34kDa
Observed MW:35kDa
Synonyms:PEX2, PAF1, PBD5A, PBD5B, PMP3, PMP35, PXMP3, RNF72, ZWS3
Background:This gene encodes an integral peroxisomal membrane protein required for peroxisome biogenesis. The protein is thought to be involved in peroxisomal matrix protein import. Mutations in this gene result in one form of Zellweger syndrome and infantile Refsum disease. Alternative splicing results in multiple transcript variants encoding the same protein.
UniProt Protein Function:PXMP3: Somewhat implicated in the biogenesis of peroxisomes. Defects in PEX2 are the cause of peroxisome biogenesis disorder complementation group 5 (PBD-CG5); also known as PBD-CGF. 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 PEX2 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. Defects in PEX2 are a cause of infantile Refsum disease (IRD). IRD is a mild peroxisome biogenesis disorder (PBD). Clinical features include early onset, mental retardation, minor facial dysmorphism, retinopathy, sensorineural hearing deficit, hepatomegaly, osteoporosis, failure to thrive, and hypocholesterolemia. The biochemical abnormalities include accumulation of phytanic acid, very long chain fatty acids (VLCFA), di- and trihydroxycholestanoic acid and pipecolic acid. Belongs to the pex2/pex10/pex12 family.
UniProt Protein Details:

Protein type:Cell development/differentiation; Membrane protein, integral; Motility/polarity/chemotaxis; Membrane protein, multi-pass; Ubiquitin conjugating system

Chromosomal Location of Human Ortholog: 8q21.1

Cellular Component: integral to peroxisomal membrane; membrane; peroxisomal membrane

Molecular Function:protein binding; zinc ion binding

Biological Process: bile acid biosynthetic process; cholesterol homeostasis; fatty acid beta-oxidation; negative regulation of epithelial cell proliferation; negative regulation of fibroblast proliferation; negative regulation of transcription from RNA polymerase II promoter; neuron migration; peroxisome organization and biogenesis; protein destabilization; protein import into peroxisome matrix; regulation of cholesterol biosynthetic process; very-long-chain fatty acid metabolic process

Disease: Peroxisome Biogenesis Disorder 5a (zellweger); Peroxisome Biogenesis Disorder 5b

NCBI Summary:This gene encodes an integral peroxisomal membrane protein required for peroxisome biogenesis. The protein is thought to be involved in peroxisomal matrix protein import. Mutations in this gene result in one form of Zellweger syndrome and infantile Refsum disease. Alternative splicing results in multiple transcript variants encoding the same protein. [provided by RefSeq, Jul 2008]
UniProt Code:P28328
NCBI GenInfo Identifier:281185478
NCBI Gene ID:5828
NCBI Accession:P28328.2
UniProt Secondary Accession:P28328,Q567S6, Q9BW41,
UniProt Related Accession:P28328
Molecular Weight:34,843 Da
NCBI Full Name:Peroxisome biogenesis factor 2
NCBI Synonym Full Names:peroxisomal biogenesis factor 2
NCBI Official Symbol:PEX2  
NCBI Official Synonym Symbols:PAF1; PMP3; ZWS3; PBD5A; PBD5B; PMP35; PXMP3; RNF72  
NCBI Protein Information:peroxisome biogenesis factor 2
UniProt Protein Name:Peroxisome biogenesis factor 2
UniProt Synonym Protein Names:35 kDa peroxisomal membrane protein; Peroxin-2; Peroxisomal membrane protein 3; Peroxisome assembly factor 1; PAF-1; RING finger protein 72
Protein Family:Peroxisome biogenesis factor
UniProt Gene Name:PEX2  
UniProt Entry Name:PEX2_HUMAN
Anti-PEX2 Antibody (CAB15308)Western blot analysis of extracts of various cell lines, using PEX2 antibody (CAB15308) 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: 20s.
Anti-PEX2 Antibody (CAB15308)Immunohistochemistry of paraffin-embedded human placenta using PEX2 Rabbit pAb (CAB15308) at dilution of 1:100 (40x lens).
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