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RAPSN rabbit pAb

商品貨號： PLA020463

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： RAPSN RNF205
蛋白名稱： RAPSN
Human_gene_id： 5913
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=5913
Human_swiss_prot_no： Q13702
Human_swiss_link： https://www.uniprot.org/uniprotkb/Q13702/entry
Mouse_gene_id： 19400
Mouse_gene_link： https://www.uniprot.org/uniprot/19400
Mouse_swiss_prot_no： P12672
Mouse_swiss_link： https://www.uniprot.org/uniprotkb/P12672
特異性： This antibody detects endogenous levels of RAPSN 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;IHC-p 1:50-300
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： 45kD
功能： disease:Defects in RAPSN are a cause of congenital myasthenic syndrome type 1d (CMS1D) [MIM:608931]; also called congenital myasthenic syndrome associated with acetylcholine receptor deficiency. Congenital myasthenic syndromes (CMS) are inherited disorders of neuromuscular transmission that stem from mutations in presynaptic, synaptic, or postsynaptic proteins. Postsynaptic disorders result from mutations in proteins forming the subunits of the muscle acetylcholine receptor (AChR). The kinetic abnormalities of AChR result in either prolonged ion channel activations that underlie "slow-channel myasthenic syndromes" (SCCMS) or abbreviated channel activations that underlie the abnormally rapid decay of endplate currents in "fast-channel syndromes" (FCCMS). CMS1D is the third disorder associated with postsynaptic CMS which could result from mutations in the proteins forming the muscle AChR. Mutations underlying AChR deficiency cause a "loss of function" and show recessive inheritance.,disease:Defects in RAPSN are the cause of fetal akinesia deformation sequence (FADS) [MIM:208150]; also known as Pena-Shokeir syndrome type 1 or fetal akinesia sequence or arthrogryposis multiplex congenita with pulmonary hypoplasia. FADS is a rare condition characterized by decreased intrauterine fetal movement, congenital limb contractures, pulmonary hypoplasia, polyhydramnios and craniofacial abnormalities.,domain:A cysteine-rich region homologous to part of the regulatory domain of protein kinase C may be important in interactions of this protein with the lipid bilayer.,function:Thought to play some role in anchoring or stabilizing the nicotinic acetylcholine receptor at synaptic sites. It may link the receptor to the underlying postsynaptic cytoskeleton, possibly by direct association with actin or spectrin.,similarity:Belongs to the RAPsyn family.,similarity:Contains 1 RING-type zinc finger.,similarity:Contains 7 TPR repeats.,subcellular location:Cytoplasmic surface of postsynaptic membranes.,
相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
細(xì)胞定位： Cell membrane; Peripheral membrane protein; Cytoplasmic side. Cell junction, synapse, postsynaptic cell membrane; Peripheral membrane protein; Cytoplasmic side. Cytoplasm, cytoskeleton. Cytoplasmic surface of postsynaptic membranes.
科研貨號： PLA020463

RAPSN rabbit pAb

Catalog No PLA020463

Product information

基因名稱： RAPSN RNF205
蛋白名稱： RAPSN
Human_gene_id： 5913
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=5913
Human_swiss_prot_no： Q13702
Human_swiss_link： https://www.uniprot.org/uniprotkb/Q13702/entry
Mouse_gene_id： 19400
Mouse_gene_link： https://www.uniprot.org/uniprot/19400
Mouse_swiss_prot_no： P12672
Mouse_swiss_link： https://www.uniprot.org/uniprotkb/P12672
特異性： This antibody detects endogenous levels of RAPSN 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;IHC-p 1:50-300
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： 45kD
功能： disease:Defects in RAPSN are a cause of congenital myasthenic syndrome type 1d (CMS1D) [MIM:608931]; also called congenital myasthenic syndrome associated with acetylcholine receptor deficiency. Congenital myasthenic syndromes (CMS) are inherited disorders of neuromuscular transmission that stem from mutations in presynaptic, synaptic, or postsynaptic proteins. Postsynaptic disorders result from mutations in proteins forming the subunits of the muscle acetylcholine receptor (AChR). The kinetic abnormalities of AChR result in either prolonged ion channel activations that underlie "slow-channel myasthenic syndromes" (SCCMS) or abbreviated channel activations that underlie the abnormally rapid decay of endplate currents in "fast-channel syndromes" (FCCMS). CMS1D is the third disorder associated with postsynaptic CMS which could result from mutations in the proteins forming the muscle AChR. Mutations underlying AChR deficiency cause a "loss of function" and show recessive inheritance.,disease:Defects in RAPSN are the cause of fetal akinesia deformation sequence (FADS) [MIM:208150]; also known as Pena-Shokeir syndrome type 1 or fetal akinesia sequence or arthrogryposis multiplex congenita with pulmonary hypoplasia. FADS is a rare condition characterized by decreased intrauterine fetal movement, congenital limb contractures, pulmonary hypoplasia, polyhydramnios and craniofacial abnormalities.,domain:A cysteine-rich region homologous to part of the regulatory domain of protein kinase C may be important in interactions of this protein with the lipid bilayer.,function:Thought to play some role in anchoring or stabilizing the nicotinic acetylcholine receptor at synaptic sites. It may link the receptor to the underlying postsynaptic cytoskeleton, possibly by direct association with actin or spectrin.,similarity:Belongs to the RAPsyn family.,similarity:Contains 1 RING-type zinc finger.,similarity:Contains 7 TPR repeats.,subcellular location:Cytoplasmic surface of postsynaptic membranes.,
相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
細(xì)胞定位： Cell membrane; Peripheral membrane protein; Cytoplasmic side. Cell junction, synapse, postsynaptic cell membrane; Peripheral membrane protein; Cytoplasmic side. Cytoplasm, cytoskeleton. Cytoplasmic surface of postsynaptic membranes.
科研貨號： PLA020463

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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