Anti-PEX3 Antibody (CAB7352)
- SKU:
- CAB7352
- Product type:
- Antibody
- Reactivity:
- Human
- Mouse
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Frequently bought together:
Description
| 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 |
 | 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. |
 | Immunohistochemistry of paraffin-embedded human liver damage using PEX3 antibody (CAB7352) at dilution of 1:100 (40x lens). |
 | Immunohistochemistry of paraffin-embedded rat kidney using PEX3 antibody (CAB7352) at dilution of 1:100 (40x lens). |
 | Immunohistochemistry of paraffin-embedded mouse kidney using PEX3 antibody (CAB7352) at dilution of 1:100 (40x lens). |
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