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CLN3 Antibody (H00001201-D01P) in WB
Western Blot analysis of CLN3 expression in transfected 293T cell line (H00001201-T02) by CLN3 MaxPab polyclonal antibody.
Lane 1: CLN3 transfected lysate(47.60 KDa).
Lane 2: Non-transfected lysate.
Lane 1: CLN3 transfected lysate(47.60 KDa).
Lane 2: Non-transfected lysate.
产品信息
H00001201-D01P
产品规格
种属反应
Human,
Mouse
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
CLN3 (NP_000077.1, 1 a.a. approximately 438 a.a) full-length human protein.
偶联物
形式
Liquid
浓度
See Label
纯化类型
Affinity chromatography
保存液
PBS, pH 7.4
内含物
no preservative
保存条件
-20° C, Avoid Freeze/Thaw Cycles
运输条件
Ambient (domestic); Wet ice (international)
产品详细信息
Sequence of this protein is as follows: MGGCAGSRRR FSDSEGEETV PEPRLPLLDH QGAHWKNAVG FWLLGLCNNF SYVVMLSAAH DILSHKRTSG NQSHVDPGPT PIPHNSSSRF DCNSVSTAAV LLADILPTLV IKLLAPLGLH LLPYSPRVLV SGICAAGSFV LVAFSHSVGT SLCGVVFASI SSGLGEVTFL SLTAFYPRAV ISWWSSGTGG AGLLGALSYL GLTQAGLSPQ QTLLSMLGIP ALLLASYFLL LTSPEAQDPG GEEEAESAAR QPLIRTEAPE SKPGSSSSLS LRERWTVFKG LLWYIVPLVV VYFAEYFINQ GLFELLFFWN TSLSHAQQYR WYQMLYQAGV FASRSSLRCC RIRFTWALAL LQCLNLVFLL ADVWFGFLPS IYLVFLIILY EGLLGGAAYV NTFHNIALET SDEHREFAMA ATCISDTLGI SLSGLLALPL HDFLCQLS
靶标信息
CLN3 is a highly glycosylated, hydrophobic, 438-amino acid protein with 6 transmembrane domains. The CLN3 protein localizes to the lysosomal membrane and plays a role in lysosomal function. It may act as a chaperone involved in the folding and unfolding of other proteins, namely subunit C of the ATP synthase complex. Mutations in the CLN3 gene cause Batten disease, a recessively inherited neurodegenerative disorder of childhood caused by lysosomal accumulation of hydrophobic material, mainly ATP synthase subunit C. Batten disease is the most common form of a group of disorders known as neuronal ceroid lipofuscinoses (NCLs). Symptoms of Batten disease include progressive loss of vision, seizures, and psychomotor disturbances.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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生物信息学
蛋白别名: Batten disease protein; Battenin; ceroid-lipofuscinosis, neuronal 3; CLN 3; MGC102840; Protein CLN3
基因别名: AI323623; BTS; CLN3; JNCL
UniProt ID: (Human) Q9UP14, (Mouse) Q61124
Entrez Gene ID: (Human) 1201, (Mouse) 12752
action potential
globoside metabolic process
cellular amino acid metabolic process
ceramide metabolic process
glucosylceramide metabolic process
galactosylceramide metabolic process
sphingomyelin metabolic process
receptor-mediated endocytosis
vacuolar transport
lysosome organization
lysosomal lumen acidification
associative learning
arginine transport
macroautophagy
negative regulation of macroautophagy
protein processing
protein catabolic process
ionotropic glutamate receptor signaling pathway
lysosomal lumen pH elevation
neurotransmitter metabolic process
amyloid precursor protein catabolic process
negative regulation of apoptotic process
negative regulation of catalytic activity
negative regulation of neuron apoptotic process
negative regulation of proteolysis
vesicle transport along microtubule
neuromuscular process controlling balance
regulation of cytosolic calcium ion concentration
membrane organization
autophagosome maturation
positive regulation of protein phosphorylation
transport
amino acid transport
endocytosis
regulation of gene expression
cell migration
actin cytoskeleton organization
regulation of filopodium assembly
protein localization to membrane
protein localization to plasma membrane
