Metabolism Antibodies 2

Anti-PEX3 Antibody (CAB7352)

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SKU:
CAB7352
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-PEX3 Antibody
Antibody SKU:CAB7352
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 144-373 of human PEX3 (NP_003621.1).
Application:WB IHC
Recommended Dilution:WB 1:500 - 1:2000 IHC 1:50 - 1:200
Reactivity:Human, Mouse, Rat
Positive Samples:Jurkat, Rat liver, Rat brain
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 144-373 of human PEX3 (NP_003621.1).
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:NAAV GKNG TTIL APPD VQQQ YLSS IQHL LGDG LTEL ITVI KQAV QKVL GSVS LKHS LSLL DLEQ KLKE IRNL VEQH KSSS WINK DGSK PLLC HYMM PDEE TPLA VQAC GLSP RDIT TIKL LNET RDML ESPD FSTV LNTC LNRG FSRL LDNM AEFF RPTE QDLQ HGNS MNSL SSVS LPLA KIIP IVNG QIHS VCSE TPSH FVQD LLTM EQVK DFAA NVYE AFST PQQL EK
Gene ID:8504
Uniprot:P56589
Cellular Location:Multi-pass membrane protein, Peroxisome membrane
Calculated MW:42kDa
Observed MW:37kDa
Synonyms:PEX3, PBD10A, PBD10B, TRG18
Background:The product of this gene is involved in peroxisome biosynthesis and integrity. It assembles membrane vesicles before the matrix proteins are translocated. 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 Zellweger syndrome (ZWS).
UniProt Protein Function:PEX3: Involved in peroxisome biosynthesis and integrity. Assembles membrane vesicles before the matrix proteins are translocated. As a docking factor for PEX19, is necessary for the import of peroxisomal membrane proteins in the peroxisomes. Defects in PEX3 are the cause of peroxisome biogenesis disorder complementation group 12 (PBD-CG12); also known as PBD-CGG. 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 PEX3 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-3 family.
UniProt Protein Details:

Protein type:Transporter, ABC family; Vesicle; Membrane protein, integral; Membrane protein, multi-pass; Transporter

Chromosomal Location of Human Ortholog: 6q24.2

Cellular Component: nucleoplasm; peroxisomal membrane; integral to peroxisomal membrane; protein complex; intracellular membrane-bound organelle; membrane; endoplasmic reticulum; peroxisome; cytosol

Molecular Function:protein dimerization activity; protein binding; lipid binding; amino acid transmembrane transporter activity

Biological Process: peroxisome organization and biogenesis; protein import into peroxisome membrane; peroxisome membrane biogenesis; transmembrane transport

Disease: Peroxisome Biogenesis Disorder 10a (zellweger)

NCBI Summary:The product of this gene is involved in peroxisome biosynthesis and integrity. It assembles membrane vesicles before the matrix proteins are translocated. 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 Zellweger syndrome (ZWS). [provided by RefSeq, Oct 2008]
UniProt Code:P56589
NCBI GenInfo Identifier:3914303
NCBI Gene ID:8504
NCBI Accession:P56589.1
UniProt Secondary Accession:P56589,Q6FGP5,
UniProt Related Accession:P56589
Molecular Weight:373
NCBI Full Name:Peroxisomal biogenesis factor 3
NCBI Synonym Full Names:peroxisomal biogenesis factor 3
NCBI Official Symbol:PEX3  
NCBI Official Synonym Symbols:TRG18; PBD10A  
NCBI Protein Information:peroxisomal biogenesis factor 3; peroxin-3; peroxisomal assembly protein PEX3; transformation-related protein 18
UniProt Protein Name:Peroxisomal biogenesis factor 3
UniProt Synonym Protein Names:Peroxin-3; Peroxisomal assembly protein PEX3
Protein Family:Peroxisome biogenesis factor
UniProt Gene Name:PEX3  
UniProt Entry Name:PEX3_HUMAN
Anti-PEX3 Antibody (CAB7352)Western blot analysis of extracts of various cell lines, using PEX3 antibody (CAB7352) 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 Enhanced Kit (RM00021)._Exposure time: 90s.
Anti-PEX3 Antibody (CAB7352)Immunohistochemistry of paraffin-embedded human liver damage using PEX3 antibody (CAB7352) at dilution of 1:100 (40x lens).
Anti-PEX3 Antibody (CAB7352)Immunohistochemistry of paraffin-embedded rat kidney using PEX3 antibody (CAB7352) at dilution of 1:100 (40x lens).
Anti-PEX3 Antibody (CAB7352)Immunohistochemistry of paraffin-embedded mouse kidney using PEX3 antibody (CAB7352) at dilution of 1:100 (40x lens).
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