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Rabbit Anti-TPP1/Gold Conjugated antibody (bs-8528R-Gold)
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说 明 书: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包装/询价
产品编号 bs-8528R-Gold
英文名称 Rabbit Anti-TPP1/Gold Conjugated antibody
中文名称 胶体金标记的细胞生长抑制基因1蛋白抗体
别    名 Cell growth inhibiting gene 1 protein; Cell growth-inhibiting gene 1 protein; Ceroid lipofuscinosis neuronal 2; Ceroid lipofuscinosis neuronal 2 late infantile (Jansky Bielschowsky disease); Ceroid lipofuscinosis neuronal 2 late infantile antibody CLN 2; CLN2; CLN-2; GIG 1; GIG1; Growth inhibiting protein 1; LPIC; Lysosomal pepstatin insensitive protease; Lysosomal pepstatin-insensitive protease; TPP 1; TPP I; TPP-1; TPP-I; Tpp1; TPP1_HUMAN; TPPI; Tripeptidyl aminopeptidase; Tripeptidyl peptidase I; Tripeptidyl-peptidase 1; Tripeptidyl-peptidase I.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul(10nm  15nm  35nm
研究领域 细胞生物  神经生物学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human,  (predicted: Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
产品应用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 40kDa
性    状 Lyophilized or Liquid
浓    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human TPP1/CLN2
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存条件 Store at 2-8 ºC for 3-6 months. Avoid repeated freeze/thaw cycles.
产品介绍 background:
Lysosomal serine protease with tripeptidyl-peptidase I activity. May act as a non-specific lysosomal peptidase which generates tripeptides from the breakdown products produced by lysosomal proteinases. Requires substrates with an unsubstituted N-terminus.

Function:
Lysosomal serine protease with tripeptidyl-peptidase I activity. May act as a non-specific lysosomal peptidase which generates tripeptides from the breakdown products produced by lysosomal proteinases. Requires substrates with an unsubstituted N-terminus (By similarity).

Subunit:
Monomer.

Subcellular Location:
Lysosome. Melanosome. Identified by mass spectrometry in melanosome fractions from stage I to stage IV.

Tissue Specificity:
Detected in all tissues examined with highest levels in heart and placenta and relatively similar levels in other tissues.

Post-translational modifications:
Activated by autocatalytic proteolytical processing upon acidification. N-glycosylation is required for processing and activity.

DISEASE:
Involvement in disease: Defects in TPP1 are the cause of neuronal ceroid lipofuscinosis type 2 (CLN2) . A form of neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The lipopigment pattern seen most often in CLN2 consists of curvilinear profiles.

Similarity:
Belongs to the peptidase S53 family.

Database links:

Entrez Gene: 1200 Human

Omim: 607998 Human

SwissProt: O14773 Human

Unigene: 523454 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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