Goat PEX6 Polyclonal Antibody | anti-PEX6 antibody

Goat anti-PEX6 Antibody

Cat. Num. AAA423150

• Gene Names: PEX6; PAF2; HMLR2; PAF-2; PBD4A; PDB4B; PXAAA1
• Reactivity: Tested: Rat; Expected from sequence similarity: Human, Mouse, Rat, Pig, Dog, Cow
• Applications: ELISA, Western Blot
• Purity: Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
• Synonyms: PEX6; Polyclonal Antibody; Goat anti-PEX6 Antibody; peroxisomal biogenesis factor 6; PAF-2; PAF2; PXAAA1; peroxisomal AAA-type ATPase 1; peroxisome biogenesis factor 6; PAF2 antibody; PAF-2 antibody; peroxisomal AAA-type ATPase 1 antibody; peroxisomal biogenesis factor 6 antibody; peroxisome biogenesis factor 6 antibody; PXAAA1 antibody; PEX6 antibody; anti-PEX6 antibody

• Host: Goat
• Reactivity: Tested: Rat; Expected from sequence similarity: Human, Mouse, Rat, Pig, Dog, Cow
• Clonality: Polyclonal
• Purity/Purification: Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
• Form/Format: Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.
• Concentration: 100ug specific antibody in 200ul (varies by lot)
• Sequence: EQELLRYKRIQRK
• Sequence Length: 980

• Applicable Applications for anti-PEX6 antibody: Peptide ELISA (EIA), Western Blot (WB)
• Application Notes: Peptide ELISA: Antibody detection limit dilution 1: 128000.; Western Blot: Approx 100kDa band observed in Rat Liver lysates (calculated MW of 104kDa according to Rat NP_476466.1). Recommended concentration: 0.3-1ug/ml.
• Immunogen: Peptide with sequence C-EQELLRYKRIQRK, from the C Terminus of the protein sequence according to NP_000278.3.
• Preparation and Storage: Aliquot and store at -20 degree C. Minimize freezing and thawing.

Western Blot (WB)

((0.3ug/ml) staining of Rat Liver lysate (35ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.)

NCBI and Uniprot Product Information

• NCBI GI #: 194018488
• NCBI GeneID: 5190
• NCBI Accession #: NP_000278.3
• NCBI GenBank Nucleotide #: NM_000287.3
• NCBI Official Full Name: peroxisome biogenesis factor 6 isoform 1
• NCBI Official 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
• Protein Family: Peroxisomal ATPase
• UniProt Gene Name: PEX6
• UniProt Synonym Gene Names: PXAAA1; PAF-2
• UniProt Entry Name: PEX6_HUMAN

NCBI Description

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 Description

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.

Protein type: Membrane protein, peripheral

Chromosomal Location of Human Ortholog: 6p21.1

Cellular Component: cytoplasm; cytosol; peroxisomal membrane; 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