Rabbit Anti-TPP1/FITC Conjugated antibody

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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;

Cat:

SL8528R-FITC

Species Reactivity：

Human,(predicted: Mouse,Rat,Dog,Pig,Cow,Horse,Rabbit,Sheep,)

Immunogen：

KLH conjugated synthetic peptide derived from human TPP1/CLN2

Format：

Lyophilized or Liquid

Storage instructions：

Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

40kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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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: 240 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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