MFSD8蛋白抗体
规格：1mg/1ml
英文名： MFSD8
别名： Ceroid-lipofuscinosis neuronal protein 7; CLN7; Major facilitator superfamily domain-containing protein 8; MFSD8; MFSD8_HUMAN.
分子量： 57kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human MFSD8
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Guinea Pig, Cat,
细胞定位：
MFSD8蛋白抗体产品介绍：background: This gene encodes a ubiquitous integral membrane protein that contains a transporter domain and a major facilitator superfamily (MFS) domain. Other members of the major facilitator superfamily transport small solutes through chemiosmotic ion gradients. The substrate transported by this protein is unknown. The protein likely localizes to lysosomal membranes. Mutations in this gene are correlated with a variant form of late infantile-onset neuronal ceroid lipofuscinoses (vLINCL). [provided by RefSeq, Oct 2008] Function: May be a carrier that transport small solutes by using chemiosmotic ion gradients. Subcellular Location: Lysosome membrane. Tissue Specificity: Expressed at very low levels in all tissues tested. DISEASE: Defects in MFSD8 are the cause of neuronal ceroid lipofuscinosis type 7 (CLN7) MFSD8蛋白抗体[MIM:610951]. A form of late infantile neuronal ceroid lipofuscinosis. CNL are a clinically and genetically heterogeneous group of neurodegenerative disorders characterized by the intracellular accumulation of autofluorescent lipopigment storage material in different patterns ultrastructurally. The patterns most often observed CLN7 are mixed combinations of granular, curvilinear, fingerprint, and rectilinear profiles. The clinical MFSD8蛋白抗体course includes progressive dementia, seizures, and progressive visual failure. Similarity: Belongs to the major facilitator superfamily. Database links: Entrez Gene: 256471 Human Entrez Gene: 72175 Mouse Entrez Gene: 361939 Rat Omim: 611124 Human SwissProt: Q8NHS3 Human SwissProt: Q8BH31 Mouse Unigene: 480701 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
MFSD8蛋白抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid