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Invitrogen
CLN2 Recombinant Mouse Monoclonal Antibody (K1H013_12G7)
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CLN2 Antibody (MA564456) in IHC (P)
Immunohistochemistry (Paraffin) analysis of CLN2 in formalin-fixed paraffin-embedded placenta tissue. Samples were incubated with CLN2 recombinant monoclonal (Product # MA564456) using a dilution of 0.3 µg/mL at room temperature for 1 hr followed by Poly-peroxidase conjugated goat anti-mouse IgG. Antigen was retrieved through addition of boiling Tris/EDTA buffer pH 9 in a pressure cooker for 3 min. Endogeno... View More
产品信息
MA564456
产品规格
种属反应
Human
宿主/亚型
Mouse
/ IgG2a
Expression System
CHO cells
分类
Recombinant Monoclonal
类型
Antibody
克隆号
K1H013_12G7
抗原
Recombinant human TPP1 (aa 201-560).
偶联物
形式
Liquid
浓度
1 mg/mL
纯化类型
Protein A
保存液
PBS with 50% glycerol, 0.5% BSA
内含物
0.09% sodium azide
保存条件
Maintain refrigerated at 2-8°C for up to 1 month. For long term storage store at -20°C
运输条件
Ambient (domestic); Wet ice (international)
RRID
产品详细信息
Immunogen sequence: TPSVIRKRYN LTSQDVGSGT SNNSQACAQF LEQYFHDSDL AQFMRLFGGN FAHQASVARV VGQQGRGRAG IEASLDVQYL MSAGANISTW VYSSPGRHEG QEPFLQWLML LSNESALPHV HTVSYGDDED SLSSAYIQRV NTELMKAAAR GLTLLFASGD SGAGCWSVSG RHQFRPTFPA SSPYVTTVGG TSFQEPFLIT NEIVDYISGG GFSNVFPRPS YQEEAVTKFL SSSPHLPPSS YFNASGRAYP DVAALSDGYW VVSNRVPIPW VSGTSASTPV FGGILSLINE HRILSGRPPL GFLNPRLYQQ HGAGLFDVTR GCHESCLDEE VEGQGFCSGP GWDPVTGWGT PNFPALLKTL
Highest antigen sequence identity to the following orthologs: Mouse - 90%, Rat - 91%, Pig - 92%, Cynomolgus monkey - 98%.
靶标信息
This gene encodes a member of the sedolisin family of serine proteases. The protease functions in the lysosome to cleave N-terminal tripeptides from substrates, and has weaker endopeptidase activity. It is synthesized as a catalytically-inactive enzyme which is activated and auto-proteolyzed upon acidification. Mutations in this gene result in late-infantile neuronal ceroid lipofuscinosis, which is associated with the failure to degrade specific neuropeptides and a subunit of ATP synthase in the lysosome.
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: Cell growth-inhibiting gene 1 protein; CLN 2; GIG 1; growth-inhibiting protein 1; LPIC; lysosomal pepstatin insensitive protease; Lysosomal pepstatin-insensitive protease; MGC21297; TPP 1; TPP I; TPP-1; TPP-I; TPPI; Tripeptidyl aminopeptidase; tripeptidyl peptidase I; Tripeptidyl-peptidase 1; Tripeptidyl-peptidase I
基因别名: CLN2; GIG1; LPIC; SCAR7; TPP-1; TPP1; UNQ267/PRO304
UniProt ID: (Human) O14773
Entrez Gene ID: (Human) 1200
proteolysis
lipid metabolic process
lysosome organization
nervous system development
central nervous system development
protein catabolic process
epithelial cell differentiation
IRE1-mediated unfolded protein response
peptide catabolic process
bone resorption
neuromuscular process controlling balance
