Cell Biology Antibodies 6

Anti-PEX12 Antibody (CAB16062)

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SKU:
CAB16062
Product Type:
Antibody
Reactivity:
Human
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Cell Biology

Description

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Antibody Name:Anti-PEX12 Antibody
Antibody SKU:CAB16062
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 290-359 of human PEX12 (NP_000277.1).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human
Positive Samples:U-87MG, MCF7
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 290-359 of human PEX12 (NP_000277.1).
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:YNSD SPLL PKMK TVCP LCRK TRVN DTVL ATSG YVFC YRCV FHYV RSHQ ACPI TGYP TEVQ HLIK LYSP EN
Gene ID:5193
Uniprot:O00623
Cellular Location:Multi-pass membrane protein, Peroxisome membrane
Calculated MW:40kDa
Observed MW:41kDa
Synonyms:PEX12, PAF-3, PBD3A
Background: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
Anti-PEX12 Antibody (CAB16062)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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