CLN5 Antibody (PACO22260)
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주문- SKU:
- PACO22260
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | CLN5 Antibody (PACO22260) |
Antibody SKU: | PACO22260 |
Size: | 100ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB |
Recommended Dilutions: | ELISA:1:2000-1:10000, WB:1:500-1:3000 |
Species Reactivity: | Human |
Immunogen: | Synthesized peptide derived from internal of human CLN5. |
Form: | Liquid |
Storage Buffer: | Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
Purification Method: | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
Western blot analysis of extracts from LOVO cells, using CLN5 antibody. |
Background: | 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. |
Synonyms: | Ceroid-lipofuscinosis neuronal protein 5; Protein 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 |