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CTNS rabbit pAb

商品貨號(hào)： PLA020611

適應(yīng) 性： 人,小鼠

￥600元

規(guī)格：

40μL 100μL 200μL

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MSDS

說明書

商品描述

基因名稱： CTNS
蛋白名稱： CTNS
Human_gene_id： 1497
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=1497
Human_swiss_prot_no： O60931
Human_swiss_link： https://www.uniprot.org/uniprotkb/O60931/entry
Mouse_gene_id： 83429
Mouse_gene_link： https://www.uniprot.org/uniprot/83429
Mouse_swiss_prot_no： P57757
Mouse_swiss_link： https://www.uniprot.org/uniprotkb/P57757
特異性： This antibody detects endogenous levels of CTNS at Human/Mouse
組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
來源： Polyclonal, Rabbit,IgG
稀釋： WB 1：500-2000
純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
濃度： 1 mg/ml
儲(chǔ)存： -15°C to -25°C/1 year(Do not lower than -25°C)
說明書： 1
Msds： MSDS_Antibody.pdf
分子量： 40kD
功能： disease:Defects in CTNS are the cause of cystinosis [MIM:219800, 219900, 219750]. This autosomal recessive disorder results from defective lysosomal transport of cystine. The classical nephropathic form is characterized by renal failure at 10 years of age and other systemic complications. Milder phenotype exist such as intermediate cystinosis, with later onset of renal disease and benign or non-nephropathic cystinosis, with symptoms related only to corneal crystals and photophobia.,function:Thought to transport cystine out of lysosomes.,similarity:Belongs to the cystinosin family.,similarity:Contains 2 PQ-loop domains.,tissue specificity:Strongly expressed in pancreas, kidney (adult and fetal) and in skeletal muscle. Expressed at lower levels in placenta and heart. Weakly expressed in lung, liver and brain (adult and fetal).,
相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
細(xì)胞定位： [Isoform 1]: Lysosome membrane ; Multi-pass membrane protein . Melanosome membrane ; Multi-pass membrane protein . AP-3 complex is required for localization to the lysosome. .; [Isoform 2]: Lysosome membrane ; Multi-pass membrane protein . Cell membrane ; Multi-pass membrane protein .
組織表達(dá)： Strongly expressed in pancreas, kidney (adult and fetal), skeletal muscle, melanocytes and keratinocytes (PubMed:22649030). Expressed at lower levels in placenta and heart. Weakly expressed in lung, liver and brain (adult and fetal) (PubMed:22649030). ; [Isoform 2]: Represents 5-20 % of CTNS transcripts, with the exception of the testis that expresses both isoforms in equal proportions.
科研貨號(hào)： PLA020611

CTNS rabbit pAb

Catalog No PLA020611

Product information

基因名稱： CTNS
蛋白名稱： CTNS
Human_gene_id： 1497
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=1497
Human_swiss_prot_no： O60931
Human_swiss_link： https://www.uniprot.org/uniprotkb/O60931/entry
Mouse_gene_id： 83429
Mouse_gene_link： https://www.uniprot.org/uniprot/83429
Mouse_swiss_prot_no： P57757
Mouse_swiss_link： https://www.uniprot.org/uniprotkb/P57757
特異性： This antibody detects endogenous levels of CTNS at Human/Mouse
組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
來源： Polyclonal, Rabbit,IgG
稀釋： WB 1：500-2000
純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
濃度： 1 mg/ml
儲(chǔ)存： -15°C to -25°C/1 year(Do not lower than -25°C)
說明書： 1
Msds： MSDS_Antibody.pdf
分子量： 40kD
功能： disease:Defects in CTNS are the cause of cystinosis [MIM:219800, 219900, 219750]. This autosomal recessive disorder results from defective lysosomal transport of cystine. The classical nephropathic form is characterized by renal failure at 10 years of age and other systemic complications. Milder phenotype exist such as intermediate cystinosis, with later onset of renal disease and benign or non-nephropathic cystinosis, with symptoms related only to corneal crystals and photophobia.,function:Thought to transport cystine out of lysosomes.,similarity:Belongs to the cystinosin family.,similarity:Contains 2 PQ-loop domains.,tissue specificity:Strongly expressed in pancreas, kidney (adult and fetal) and in skeletal muscle. Expressed at lower levels in placenta and heart. Weakly expressed in lung, liver and brain (adult and fetal).,
相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
細(xì)胞定位： [Isoform 1]: Lysosome membrane ; Multi-pass membrane protein . Melanosome membrane ; Multi-pass membrane protein . AP-3 complex is required for localization to the lysosome. .; [Isoform 2]: Lysosome membrane ; Multi-pass membrane protein . Cell membrane ; Multi-pass membrane protein .
組織表達(dá)： Strongly expressed in pancreas, kidney (adult and fetal), skeletal muscle, melanocytes and keratinocytes (PubMed:22649030). Expressed at lower levels in placenta and heart. Weakly expressed in lung, liver and brain (adult and fetal) (PubMed:22649030). ; [Isoform 2]: Represents 5-20 % of CTNS transcripts, with the exception of the testis that expresses both isoforms in equal proportions.
科研貨號(hào)： PLA020611

Hunan UPT Biotechnology Co.,Ltd

Website：m.tjdqdt.com Servive hotline :4006916686

E-mail：service@uptbio.com

Address：

Room 402, Building 13, Xinggong International Industrial Park, 100 Guyuan Road, Yuelu District, Changsha City, Hunan Province, China.

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