Name: Anti-PEX10 Antibody (CAB16949)
Brand: Metabolism Antibodies 2
SKU: CAB16949
Availability: OutOfStock

Antibody Name:	Anti-PEX10 Antibody
Antibody SKU:	CAB16949
Antibody Size:	20uL, 50uL, 100uL
Application:	WB
Reactivity:	Human, Mouse
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 10-160 of human PEX10 (NP_002608.1).

Application:	WB
Recommended Dilution:	WB 1:500 - 1:2000
Reactivity:	Human, Mouse
Positive Samples:

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 10-160 of human PEX10 (NP_002608.1).
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:	EVIR AAQK DEYY RGGL RSAA GGAL HSLA GARK WLEW RKEV ELLS DVAY FGLT TLAG YQTL GEEY VSII QVDP SRIH VPSS LRRG VLVT LHAV LPYL LDKA LLPL EQEL QADP DSGR PLQG SLGP GGRG CSGA RRWM RHHT ATLT EQQR RAL
Gene ID:	5192
Uniprot:	O60683
Cellular Location:
Calculated MW:
Observed MW:	Refer to figures

Synonyms:	PEX10, NALD, PBD6A, PBD6B, RNF69
Background:	This gene encodes a protein involved in import of peroxisomal matrix proteins. This protein localizes to the peroxisomal membrane. Mutations in this gene result in phenotypes within the Zellweger spectrum of peroxisomal biogenesis disorders, ranging from neonatal adrenoleukodystrophy to Zellweger syndrome. Alternative splicing results in two transcript variants encoding different isoforms.

UniProt Protein Function:	PEX10: Somewhat implicated in the biogenesis of peroxisomes. Defects in PEX10 are the cause of peroxisome biogenesis disorder complementation group 7 (PBD-CG7); also known as PBD-CGB. 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 PEX10 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 PEX10 are a cause of adrenoleukodystrophy neonatal (NALD). NALD is a peroxisome biogenesis disorder (PBD) characterized by the accumulation of very long- chain fatty acids, adrenal insufficiency and mental retardation. Belongs to the pex2/pex10/pex12 family. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:	Protein type:Ubiquitin conjugating system; Ubiquitin ligase; Ligase; EC 6.3.2.- Chromosomal Location of Human Ortholog: 1p36.32 Cellular Component: integral to peroxisomal membrane; intracellular; peroxisomal membrane; peroxisome Molecular Function:protein binding; protein C-terminus binding; zinc ion binding Biological Process: peroxisome organization and biogenesis; protein import into peroxisome matrix Disease: Peroxisome Biogenesis Disorder 6a (zellweger); Peroxisome Biogenesis Disorder 6b
NCBI Summary:	This gene encodes a protein involved in import of peroxisomal matrix proteins. This protein localizes to the peroxisomal membrane. Mutations in this gene result in phenotypes within the Zellweger spectrum of peroxisomal biogenesis disorders, ranging from neonatal adrenoleukodystrophy to Zellweger syndrome. Alternative splicing results in two transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008]
UniProt Code:	O60683
NCBI GenInfo Identifier:	3914299
NCBI Gene ID:	5192
NCBI Accession:	O60683.1
UniProt Secondary Accession:	O60683,Q5T095, Q9BW90, B3KWD8,
UniProt Related Accession:	O60683
Molecular Weight:	39,214 Da
NCBI Full Name:	Peroxisome biogenesis factor 10
NCBI Synonym Full Names:	peroxisomal biogenesis factor 10
NCBI Official Symbol:	PEX10
NCBI Official Synonym Symbols:	NALD; PBD6A; PBD6B; RNF69
NCBI Protein Information:	peroxisome biogenesis factor 10
UniProt Protein Name:	Peroxisome biogenesis factor 10
UniProt Synonym Protein Names:	Peroxin-10; Peroxisomal biogenesis factor 10; Peroxisome assembly protein 10; RING finger protein 69
Protein Family:	Peroxisome biogenesis factor
UniProt Gene Name:	PEX10
UniProt Entry Name:	PEX10_HUMAN

Western blot analysis of extracts of various cell lines, using PEX10 antibody (CAB16949) 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: 90s.

Antibodies

Anti-PEX10 Antibody (CAB16948)

PEX10 Antibody (PACO04578)

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)