Peroxin 7 Polyclonal Antibody

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商品货号： PLA017579

适应性： 人,小鼠,大鼠

￥600元

规格：

40μL 100μL 200μL

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MSDS

说明书

商品描述

基因名称： PEX7
蛋白名称： Peroxisomal targeting signal 2 receptor
Human_gene_id： 5191
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=5191
Human_swiss_prot_no： O00628
Human_swiss_link： http://www.uniprot.org/uniprotkb/O00628/entry
Mouse_gene_id： 18634
Mouse_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=18634
Mouse_swiss_prot_no： P97865
Mouse_swiss_link： http://www.uniprot.org/uniprot/P97865
特异性： Peroxin 7 Polyclonal Antibody detects endogenous levels of Peroxin 7 protein.
组成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
来源： Polyclonal, Rabbit,IgG
稀释： WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:40000.. IF 1:50-200
纯化工艺： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
浓度： 1 mg/ml
储存： -15°C to -25°C/1 year(Do not lower than -25°C)
说明书： 1
Msds： MSDS_Antibody.pdf
其他名称： PEX7; PTS2R; Peroxisomal targeting signal 2 receptor; PTS2 receptor; Peroxin-7
实测条带： 40kD
功能： disease:Defects in PEX7 are a cause of Refsum disease (RD) [MIM:266500]; also known as phytanic acid oxidase deficiency. RD is clinically characterized by a tetrad of abnormalities: retinitis pigmentosa, peripheral neuropathy, cerebellar ataxia, and elevated protein levels in the cerebrospinal fluid (CSF). Patients exhibit accumulation of the branched-chain fatty acid, phytanic acid, in blood and tissues. Less constant features are nerve deafness, anosmia, skeletal abnormalities, ichthyosis, cataracts and cardiac impairment. Manifestations of the disease appear in the second or third decade of life.,disease:Defects in PEX7 are the cause of peroxisome biogenesis disorder complementation group 11 (PBD-CG11) [MIM:601757]. 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 13 distinct genetic groups as concluded from complementation studies.,disease:Defects in PEX7 are the cause of rhizomelic chondrodysplasia punctata type 1 (RCDP1) [MIM:215100]. RCDP1 is characterized by rhizomelic shortening of femur and humerus, vertebral disorders, cataract, cutaneous lesions and severe mental retardation.,function:Binds to the N-terminal PTS2-type peroxisomal targeting signal and plays an essential role in peroxisomal protein import.,similarity:Belongs to the WD repeat peroxin-7 family.,similarity:Contains 6 WD repeats.,subunit:Interacts with PEX5.,tissue specificity:Ubiquitous. Highest expression in pancreas, skeletal muscle and heart.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Peroxisome . Cytoplasm .
组织表达： Ubiquitous. Highest expression in pancreas, skeletal muscle and heart.
科研货号： PLA017579

Peroxin 7 Polyclonal Antibody

Catalog No PLA017579

Product information

基因名称： PEX7
蛋白名称： Peroxisomal targeting signal 2 receptor
Human_gene_id： 5191
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=5191
Human_swiss_prot_no： O00628
Human_swiss_link： http://www.uniprot.org/uniprotkb/O00628/entry
Mouse_gene_id： 18634
Mouse_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=18634
Mouse_swiss_prot_no： P97865
Mouse_swiss_link： http://www.uniprot.org/uniprot/P97865
特异性： Peroxin 7 Polyclonal Antibody detects endogenous levels of Peroxin 7 protein.
组成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
来源： Polyclonal, Rabbit,IgG
稀释： WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:40000.. IF 1:50-200
纯化工艺： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
浓度： 1 mg/ml
储存： -15°C to -25°C/1 year(Do not lower than -25°C)
说明书： 1
Msds： MSDS_Antibody.pdf
其他名称： PEX7; PTS2R; Peroxisomal targeting signal 2 receptor; PTS2 receptor; Peroxin-7
实测条带： 40kD
功能： disease:Defects in PEX7 are a cause of Refsum disease (RD) [MIM:266500]; also known as phytanic acid oxidase deficiency. RD is clinically characterized by a tetrad of abnormalities: retinitis pigmentosa, peripheral neuropathy, cerebellar ataxia, and elevated protein levels in the cerebrospinal fluid (CSF). Patients exhibit accumulation of the branched-chain fatty acid, phytanic acid, in blood and tissues. Less constant features are nerve deafness, anosmia, skeletal abnormalities, ichthyosis, cataracts and cardiac impairment. Manifestations of the disease appear in the second or third decade of life.,disease:Defects in PEX7 are the cause of peroxisome biogenesis disorder complementation group 11 (PBD-CG11) [MIM:601757]. 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 13 distinct genetic groups as concluded from complementation studies.,disease:Defects in PEX7 are the cause of rhizomelic chondrodysplasia punctata type 1 (RCDP1) [MIM:215100]. RCDP1 is characterized by rhizomelic shortening of femur and humerus, vertebral disorders, cataract, cutaneous lesions and severe mental retardation.,function:Binds to the N-terminal PTS2-type peroxisomal targeting signal and plays an essential role in peroxisomal protein import.,similarity:Belongs to the WD repeat peroxin-7 family.,similarity:Contains 6 WD repeats.,subunit:Interacts with PEX5.,tissue specificity:Ubiquitous. Highest expression in pancreas, skeletal muscle and heart.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Peroxisome . Cytoplasm .
组织表达： Ubiquitous. Highest expression in pancreas, skeletal muscle and heart.
科研货号： PLA017579

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