CLN5 Antibody, FITC conjugated (PACO56762)
- SKU:
- PACO56762
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- FITC
Frequently bought together:
Description
抗体名: | CLN5 Antibody, FITC conjugated (PACO56762) |
抗体コード: | PACO56762 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human Ceroid-lipofuscinosis neuronal protein 5 protein (42-181AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | FITC |
バックグラウンド: | Plays a role in influencing the retrograde trafficking of lysosomal sorting receptors SORT1 and IGF2R from the endosomes to the trans-Golgi network by controlling the recruitment of retromer complex to the endosomal membrane. Regulates the localization and activation of RAB7A which is required to recruit the retromer complex to the endosomal membrane. |
シノニム: | Ceroid-lipofuscinosis neuronal protein 5 (Protein CLN5) [Cleaved into: Ceroid-lipofuscinosis neuronal protein 5, secreted form], CLN5 |
UniProt Protein Function: | CLN5: Defects in CLN5 are the cause of neuronal ceroid lipofuscinosis type 5 (CLN5); also known as Finnish variant late-infantile neuronal ceroid lipofuscinosis (vLINCL). 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 patterns observed most often in neuronal ceroid lipofuscinosis type 5 comprise mixed combinations of granular, curvilinear, and fingerprint profiles. Belongs to the CLN5 family. |
UniProt Protein Details: | Protein type:Membrane protein, integral Chromosomal Location of Human Ortholog: 13q22.3 Cellular Component: endoplasmic reticulum; Golgi apparatus; integral to membrane; lysosomal membrane; lysosome; perinuclear region of cytoplasm Molecular Function:mannose binding; protein binding Biological Process: brain development; lysosomal lumen acidification; neurogenesis; retrograde transport, endosome to Golgi; signal peptide processing Disease: Ceroid Lipofuscinosis, Neuronal, 5 |
NCBI Summary: | This gene is one of eight which have been associated with neuronal ceroid lipofuscinoses (NCL). Also referred to as Batten disease, NCL comprises a class of autosomal recessive, neurodegenerative disorders affecting children. The genes responsible likely encode proteins involved in the degradation of post-translationally modified proteins in lysosomes. The primary defect in NCL disorders is thought to be associated with lysosomal storage function.[provided by RefSeq, Oct 2008] |
UniProt Code: | O75503 |
NCBI GenInfo Identifier: | 187608866 |
NCBI Gene ID: | 1203 |
NCBI Accession: | O75503.2 |
UniProt Secondary Accession: | O75503,B3KQK7, |
UniProt Related Accession: | O75503 |
Molecular Weight: | 41kDa |
NCBI Full Name: | Ceroid-lipofuscinosis neuronal protein 5 |
NCBI Synonym Full Names: | ceroid-lipofuscinosis, neuronal 5 |
NCBI Official Symbol: | CLN5 |
NCBI Official Synonym Symbols: | NCL |
NCBI Protein Information: | ceroid-lipofuscinosis neuronal protein 5 |
UniProt Protein Name: | Ceroid-lipofuscinosis neuronal protein 5 |
Protein Family: | Cln5-like protein |
UniProt Gene Name: | CLN5 |