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NPC1 rabbit pAb

商品貨號： PLA020796

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： NPC1
蛋白名稱： NPC1
Human_gene_id： 4864
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=4864
Human_swiss_prot_no： O15118
Human_swiss_link： https://www.uniprot.org/uniprotkb/O15118/entry
Mouse_swiss_prot_no： O35604
Mouse_swiss_link： https://www.uniprot.org/uniprotkb/O35604
特異性： This antibody detects endogenous levels of NPC1 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： 141kD
功能： disease:Defects in NPC1 are the cause of Niemann-Pick disease type C1 (NPC1) [MIM:257220]. NPC1 is an autosomal recessive lipid storage disorder, which affects particularly the brain, liver and spleen, and which is characterized by lysosomal accumulation of low density lipoprotein derived cholesterol. Clinical features include variable hepatosplenomegaly and severe progressive neurological dysfunction such as ataxia, dystonia and dementia. The age of onset can vary from infancy to late adulthood.,disease:Defects in NPC1 are the cause of Niemann-Pick disease type D (NPD) [MIM:257220]; also known as Niemann-Pick disease without sphingomyelinase deficiency, or Nova Scotian type. Because of evidence from biochemical changes, lack of complementation, and linkage mapping to the same chromosome site, NPD and NPC1 are considered to be allelic disorders.,domain:A cysteine-rich N-terminal domain and a C-terminal domain containing a di-leucine motif necessary for lysosomal targeting are critical for mobilization of cholesterol from lysosomes.,function:Involved in the intracellular trafficking of cholesterol. May play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals.,PTM:Glycosylated.,similarity:Belongs to the patched family.,similarity:Contains 1 SSD (sterol-sensing) domain.,
相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
細胞定位： Late endosome membrane ; Multi-pass membrane protein . Lysosome membrane ; Multi-pass membrane protein .
科研貨號： PLA020796

NPC1 rabbit pAb

Catalog No PLA020796

Product information

基因名稱： NPC1
蛋白名稱： NPC1
Human_gene_id： 4864
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=4864
Human_swiss_prot_no： O15118
Human_swiss_link： https://www.uniprot.org/uniprotkb/O15118/entry
Mouse_swiss_prot_no： O35604
Mouse_swiss_link： https://www.uniprot.org/uniprotkb/O35604
特異性： This antibody detects endogenous levels of NPC1 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： 141kD
功能： disease:Defects in NPC1 are the cause of Niemann-Pick disease type C1 (NPC1) [MIM:257220]. NPC1 is an autosomal recessive lipid storage disorder, which affects particularly the brain, liver and spleen, and which is characterized by lysosomal accumulation of low density lipoprotein derived cholesterol. Clinical features include variable hepatosplenomegaly and severe progressive neurological dysfunction such as ataxia, dystonia and dementia. The age of onset can vary from infancy to late adulthood.,disease:Defects in NPC1 are the cause of Niemann-Pick disease type D (NPD) [MIM:257220]; also known as Niemann-Pick disease without sphingomyelinase deficiency, or Nova Scotian type. Because of evidence from biochemical changes, lack of complementation, and linkage mapping to the same chromosome site, NPD and NPC1 are considered to be allelic disorders.,domain:A cysteine-rich N-terminal domain and a C-terminal domain containing a di-leucine motif necessary for lysosomal targeting are critical for mobilization of cholesterol from lysosomes.,function:Involved in the intracellular trafficking of cholesterol. May play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals.,PTM:Glycosylated.,similarity:Belongs to the patched family.,similarity:Contains 1 SSD (sterol-sensing) domain.,
相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
細胞定位： Late endosome membrane ; Multi-pass membrane protein . Lysosome membrane ; Multi-pass membrane protein .
科研貨號： PLA020796

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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