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Rabbit Anti-Neuroserpin/FITC Conjugated antibody
background:
Neuroserpin is an axonally secreted glycoprotein in the central nervous system (CNS) that belongs to the family of protease inhibitors known as serpins (1,2). Neuroserpin is a serine-protease inhibitor (3) that forms SDS-stable complexes with tissue plasminogen activator (tPA), urokinase and plasmin, but not thrombin (2,4,5). Neuroserpin is expressed in the neocortex, the hippocampal formation, the olfactory bulb and the amygdala in the adult CNS, and it is expressed in the cerebellum, the granule cells and a subgroup of Purkinje cells in the developing embryo (2). tPA expression has been linked to "neuronal plasticity", either in the developing embryo CNS (2,4) or in cases of synpatic remodeling or long-term potentiation (6). Overexpression of tPA may promote neuronal cell death (6). Mutations in the gene which codes for neuroserpin are linked to hereditary dementia (7). Intracerebral administration of neuroserpin after stroke decreases stroke volume and diminishes the apoptotic features of the resulting ischemic penumbra (6).
Function:
Serine protease inhibitor that inhibits plasminogen activators and plasmin but not thrombin. May be involved in the formation or reorganization of synaptic connections as well as for synaptic plasticity in the adult nervous system. May protect neurons from cell damage by tissue-type plasminogen activator.
Subcellular Location:
Secreted.
Tissue Specificity:
Predominantly expressed in the brain.
DISEASE:
Defects in SERPINI1 are the cause of familial encephalopathy with neuroserpin inclusion bodies (FEN1B) [MIM:604218]. FEN1B is characterized clinically as an autosomal dominantly inherited dementia, histologically by unique neuronal inclusion bodies and biochemically by polymers of neuroserpin.
Similarity:
Belongs to the serpin family.
Database links:
Entrez Gene: 5274 Human
Entrez Gene: 20713 Mouse
Entrez Gene: 116459 Rat
Omim: 602445 Human
SwissProt: Q99574 Human
SwissProt: O35684 Mouse
SwissProt: Q9JLD2 Rat
Unigene: 478153 Human
Unigene: 41560 Mouse
Unigene: 52632 Rat
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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