Anti-PEX19 Rabbit pAb
- 100ug (NP33050) Please inquiry
Antibody type:Primary antibody
Label:Unconjugated
Modification:Unmodification
Isotype:Rabbit IgG
Host:Rabbit
Application:WB,IHC
Purify method:Affinity purified
Species:Human,Mouse
Gene Name:PEX19
Synonyms:PXF; HK33; PMP1; PMPI; PXMP1; PBD12A; D1S2223E
Gene Synonyms:
Gene Full Name:peroxisomal biogenesis factor 19
Gene Infomation:This gene is necessary for early peroxisomal biogenesis. It acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). 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. These disorders have at least 14 complementation groups, with more than one phenotype being observed for some 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), as well as peroxisome biogenesis disorder complementation group 14 (PBD-CG14), which is also known as PBD-CGJ. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2010]
Antigen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-299 of human PEX19 (NP_002848.1).
Antigen Synonyms:Peroxisomal biogenesis factor 19
Clonality:Polyclonal antibody
Source:Human
Reaction:
Form:Liquid
Tested Applications:
- Western blot (1:100 to 1:500)
- Immunofluorescence (1:50 to 1:400)
- Immunohistochemistry (1:200 to 1:500)
- Flow cytometry analysis (1:200 to 1:500)
- Enzyme-linked Immunosorbent Assay (1:100-1:5000)
Clone:
Dilution:WB1:500 -1:2000; IHC1:50 -1:200(Optimal dilutions should be determined by the end user)
Mole Mass:37kDa
Location:Cytoplasm, Membrane, Peroxisome
Concentration:
Sequence Similarity:
Gene Id:
SwissProt ID:P40855
Unigene:5824
Nucleotide Accession:
Tissue specificity:Ubiquitously expressed. Isoform 1 is strongly predominant in all tissues except in utero where isoform 2 is the main form.
Storage:Store at -20°C. Avoid freeze / thaw cycles.
Buffer condition:PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Background:
Molar Function:Necessary for early peroxisomal biogenesis. Acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). Binds and stabilizes newly synthesized PMPs in the cytoplasm by interacting with their hydrophobic membrane-spanning domains, and targets them to the peroxisome membrane by binding to the integral membrane protein PEX3. Excludes CDKN2A from the nucleus and prevents its interaction with MDM2, which results in active degradation of TP53.
Western Blot:Stability: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.
Storage: Store at 4°C for frequent use. Stored at -20°C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.
Notes: For In vitro laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.