Family main features
SERPINI1 (serpin family I member 1) is a gene encoding the neuroserpin protein, a member of the serpin (serine protease inhibitor) superfamily. Unlike many other serpins, neuroserpin is primarily expressed in the central nervous system, where it plays a critical role in regulating proteolytic activity, particularly by inhibiting tissue-type plasminogen activator (tPA). The balance between neuroserpin and tPA is crucial for neural development, synaptic plasticity, and maintaining the integrity of neuronal tissues.
SERPINI1 mutations are associated with certain neurodegenerative diseases, notably familial encephalopathy with neuroserpin inclusion bodies (FENIB), a condition characterized by the accumulation of misfolded neuroserpin proteins in neurons. This leads to neurodegeneration, progressive cognitive decline, and other neurological impairments.
Neuroserpin, the protein product of SERPINI1, is a glycoprotein made up of 410 amino acids, with a molecular weight of approximately 46 kDa. Like other members of the serpin family, neuroserpin adopts a conserved serpin fold, consisting of three beta-sheets and several alpha-helices. However, neuroserpin has unique structural features that enable it to function specifically in the nervous system.
Key structural components of neuroserpin include:
Unlike other serpins, neuroserpin has adapted to function optimally within the unique environment of the nervous system. Its structure is highly specialized to regulate proteolysis in the brain, preventing excessive degradation of extracellular matrix components that are essential for synaptic function and plasticity.
SERPINI1 belongs to the I family of serpins, which primarily function in non-hepatic tissues. Neuroserpin is the most well-characterized member of this family, with its primary role being the regulation of proteolytic activity in the brain. While neuroserpin has not been classified into subtypes, mutations in the SERPINI1 gene can give rise to different forms of misfolded proteins that vary in their propensity to form aggregates, leading to varying severity of neurological disorders.
Neuroserpin plays a crucial role in regulating proteolytic activity in the nervous system. Its primary target is tissue-type plasminogen activator (tPA), a serine protease involved in the conversion of plasminogen to plasmin. Plasmin is a powerful proteolytic enzyme that breaks down extracellular matrix proteins and is involved in processes such as fibrinolysis and tissue remodeling. In the brain, the balance between neuroserpin and tPA is essential for maintaining the integrity of the neural extracellular matrix, which is important for synaptic plasticity, neuronal survival, and brain development.
Mutations in the SERPINI1 gene lead to the production of misfolded neuroserpin proteins, which can aggregate and form intracellular inclusion bodies in neurons. These aggregates disrupt cellular function, leading to progressive neurodegeneration and a condition known as familial encephalopathy with neuroserpin inclusion bodies (FENIB). FENIB is characterized by the following clinical features:
Research into the therapeutic targeting of neuroserpin has focused on both the treatment of FENIB and the potential neuroprotective applications of enhancing neuroserpin function. Several approaches are being explored:
SERPINI1, encoding the neuroserpin protein, plays a vital role in regulating proteolytic activity in the nervous system by inhibiting tissue-type plasminogen activator (tPA). This regulation is essential for maintaining synaptic plasticity, protecting neurons from proteolytic damage, and supporting normal brain function. Mutations in SERPINI1 lead to the accumulation of misfolded neuroserpin, resulting in neurodegenerative diseases such as FENIB. Understanding the structure, function, and clinical implications of SERPINI1 is crucial for developing therapeutic strategies to treat or mitigate neuroserpin-related disorders and protect brain health.
serpin family I member 1 (SERPINI1)
PI12,HNS-S1,HNS-S2,neuroserpin,Peptidase inhibitor 12,Serpin I1
En la tabla siguiente se muestra una comparativa de todos los reactivos disponibles en nuestro catálogo (Proteins and Peptides, Primary Antibodies, CLIA Kits, ELISA Kits) relacionados con SERPINI1 - serpin family I member 1
Se muestran ordenados por categorías para poder comparar cómodamente sus características principales. Esta tabla, que contiene un enlace con la ficha de cada producto, es exportable a Excel.
Esta página contiene 15 reactivos de las marcas (Abbexa, FineTest) que se corresponden con tu busqueda
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provider | Code | reference | name | reactivity | sample type | assay type | test range | sensitivity | price | size 1 | uniprot id | status |
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Abbexa | SERPINI1 | abx494241 | Human Neuroserpin (NSP) CLIA Kit | Human | Serum, plasma and other biological fluids. | Sandwich | 0.781 ng/ml - 50 ng/ml | < 0.34 ng/ml | 812.5 | 96 tests | RUO | |
Abbexa | SERPINI1 | abx535107 | Mouse Neuroserpin (SERPINI1) ELISA Kit | Mouse | Serum,Plasma,Tissue homogenates,Cell lysates,Other biological fluids | 0.156 ng/ml - 10 ng/ml | 687.5 | 96 tests | O35684 | RUO | ||
Abbexa | SERPINI1 | abx535108 | Rat Neuroserpin (SERPINI1) ELISA Kit | Rat | Serum,Plasma,Tissue homogenates,Cell lysates,Other biological fluids | 0.156 ng/ml - 10 ng/ml | 687.5 | 96 tests | Q9JLD2 | RUO |
provider | Code | reference | name | reactivity | clonality | host | immunogen target | isotype | conjugation | tested applications | price | size 1 | uniprot id | status |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Abbexa | SERPINI1 | abx025697 | Neuroserpin (SERPINI1) Antibody | Human | Polyclonal | Rabbit | Neuroserpin (SERPINI1) | Unconjugated | ELISA, WB, IHC, IF/ICC, FCM | 281.25 | 80 µl | Q99574 | RUO | |
Abbexa | SERPINI1 | abx317629 | Neuroserpin (SERPINI1) Antibody (FITC) | Human | Polyclonal | Rabbit | Neuroserpin (SERPINI1) | FITC | 162.5 | 20 µg | Q99574 | RUO | ||
Abbexa | SERPINI1 | abx317628 | Neuroserpin (SERPINI1) Antibody (HRP) | Human | Polyclonal | Rabbit | Neuroserpin (SERPINI1) | HRP | ELISA | 162.5 | 20 µg | Q99574 | RUO | |
Abbexa | SERPINI1 | abx317630 | Neuroserpin (SERPINI1) Antibody (Biotin) | Human | Polyclonal | Rabbit | Neuroserpin (SERPINI1) | Biotin | ELISA | 162.5 | 20 µg | Q99574 | RUO | |
Abbexa | SERPINI1 | abx433030 | Neuroserpin (SERPINI1) Antibody | Human | Polyclonal | Goat | Neuroserpin (SERPINI1) | Unconjugated | P-ELISA, WB, IHC | 387.5 | 200 µl | RUO | ||
Abbexa | SERPINI1 | abx301904 | Neuroserpin (SERPINI1) Antibody | Human | Polyclonal | Rabbit | Neuroserpin (SERPINI1) | Unconjugated | ELISA, IHC | 162.5 | 20 µg | Q99574 | RUO | |
Abbexa | SERPINI1 | abx115478 | Neuroserpin (SERPINI1) Antibody | Human | Polyclonal | Rabbit | Neuroserpin (SERPINI1) | Unconjugated | ELISA, WB | 612.5 | 100 µl | Q99574 | RUO | |
Abbexa | SERPINI1 | abx235681 | Neuroserpin (SERPINI1) Antibody | Human | Polyclonal | Rabbit | Neuroserpin (SERPINI1) | Unconjugated | ELISA, WB | 350 | 100 µg | Q99574 | RUO |
provider | Code | reference | name | origin | expression | host | conjugation | tested applications | price | size 1 | uniprot id | status |
---|---|---|---|---|---|---|---|---|---|---|---|---|
FineTest | SERPINI1 | P6488 | Recombinant Human Neuroserpin | Human | Recombinant | E.Coli | Western Blot,ELISA | 50μg | Q99574 | RUO | ||
Abbexa | SERPINI1 | abx692199 | Mouse Neuroserpin (SerpinI1) Protein | Mouse | Recombinant | HEK293 cells | SDS-PAGE | 837.5 | 50 µg | O35684 | RUO | |
Abbexa | SERPINI1 | abx262659 | Serpin Peptidase Inhibitor, Clade I Member 1 Protein | Recombinant | Unconjugated | SDS-PAGE | 225 | 2 µg | Q99574 | RUO | ||
Abbexa | SERPINI1 | abx261371 | Serpin Peptidase Inhibitor, Clade I Member 1 Protein | Recombinant | Unconjugated | SDS-PAGE | 225 | 5 µg | Q99574 | RUO |
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