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Peroxin 19 Rabbit pAb

-YT3674

Catalog: YT3674
Size
Price
Status
Qty.
200μL
$450.00
In stock

0

100μL
$280.00
In stock

0

40μL
$150.00
In stock

0

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Main Information
Target

Peroxin 19

Host Species

Rabbit

Reactivity

Human, Rat, Mouse,

Applications

WB, IHC, IF, ELISA

MW

33kD (Observed)

Conjugate/Modification


Unmodified

Detailed Information
Recommended Dilution Ratio
WB 1:500-1:2000; IHC 1:100-1:300; ELISA 1:5000; IF 1:50-200
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Specificity
Peroxin 19 Polyclonal Antibody detects endogenous levels of Peroxin 19 protein.
Purification
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Storage
-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
1 mg/ml
MW(Observed)
33kD
Modification
Unmodified
Clonality
Polyclonal
Isotype
IgG
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Antigen&Target Information
Immunogen:
The antiserum was produced against synthesized peptide derived from human PEX19. AA range:219-268
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Specificity:
Peroxin 19 Polyclonal Antibody detects endogenous levels of Peroxin 19 protein.
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Gene Name:
PEX19
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Protein Name:
Peroxisomal biogenesis factor 19
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Other Name:
PEX19 ;
HK33 ;
PXF ;
OK/SW-cl.22 ;
Peroxisomal biogenesis factor 19 ;
33 kDa housekeeping protein ;
Peroxin-19 ;
Peroxisomal farnesylated protein
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Database Link:
Organism Gene ID SwissProt
Human 5824; P40855;
Mouse Q8VCI5;
Background:
peroxisomal biogenesis factor 19(PEX19) Homo sapiens 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
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Function:
Alternative products:Experimental confirmation may be lacking for some isoforms,Disease:Defects in PEX19 are a cause of Zellweger syndrome (ZWS) [MIM:214100]. 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.,Disease:Defects in PEX19 are the cause of peroxisome biogenesis disorder complementation group 14 (PBD-CG14) [MIM:600279]; also known as PBD-CGJ. 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.,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.,similarity:Belongs to the peroxin-19 family.,subcellular location:Mainly cytoplasmic. Some fraction membrane-associated to the outer surface of peroxisomes.,subunit:Interacts with a broad range of peroxisomal membrane proteins, including PEX3, PEX10, PEX11A, PEX11B, PEX12, PEX13, PEX14 and PEX16, PXMP2/PMP22, PXMP4/PMP24, SLC25A17/PMP34, ABCD1/ALDP, ABCD2/ALDRP, and ABCD3/PMP70. Also interacts with the tumor suppressor CDKN2A/p19ARF.,tissue specificity:Ubiquitously expressed. Isoform 1 is strongly predominant in all tissues except in utero where isoform 2 is the main form.,
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Cellular Localization:
Cytoplasm . Peroxisome membrane ; Lipid-anchor ; Cytoplasmic side . Mainly cytoplasmic. Some fraction membrane-associated to the outer surface of peroxisomes. .
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Tissue Expression:
Ubiquitously expressed. Isoform 1 is strongly predominant in all tissues except in utero where isoform 2 is the main form.
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Research Areas:
>>Peroxisome
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Catalog: YT3674
Size
Price
Status
Qty.
200μL
$450.00
In stock

0

100μL
$280.00
In stock

0

40μL
$150.00
In stock

0

Add to cart

Collected

Collect

Customized Service

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