CLN8 Antibody (PACO61173)
- SKU:
- PACO61173
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
抗体名: | CLN8 Antibody (PACO61173) |
抗体コード: | PACO61173 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA, IHC |
推奨される希釈: | ELISA:1:2000-1:10000, IHC:1:20-1:200 |
反応性: | Human |
免疫原: | Recombinant Human Protein CLN8 protein (247-286AA) |
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: | Non-conjugated |
![]() | IHC image of PACO61173 diluted at 1:100 and staining in paraffin-embedded human prostate cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. |
バックグラウンド: | Could play a role in cell proliferation during neuronal differentiation and in protection against cell death. |
シノニム: | Protein CLN8, CLN8, C8orf61 |
UniProt Protein Function: | CLN8: Could play a role in cell proliferation during neuronal differentiation and in protection against cell death. Defects in CLN8 are the cause of neuronal ceroid lipofuscinosis type 8 (CLN8). A form of neuronal ceroid lipofuscinosis with onset in childhood. 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 8 comprise mixed combinations of granular, curvilinear, and fingerprint profiles. Defects in CLN8 are the cause of neuronal ceroid lipofuscinosis type 8 Northern epilepsy variant (CLN8NE). A form of neuronal ceroid lipofuscinosis clinically characterized by epilepsy that presents between 5 and 10 years of age with frequent tonic-clonic seizures followed by progressive mental retardation. Visual loss is not a prominent feature. Intracellular accumulation of autofluorescent material results in curvilinear and granular profiles on ultrastructural analysis. |
UniProt Protein Details: | Protein type:Cell development/differentiation; Endoplasmic reticulum; Membrane protein, multi-pass; Membrane protein, integral; Apoptosis Chromosomal Location of Human Ortholog: 8p23 Cellular Component: endoplasmic reticulum; ER-Golgi intermediate compartment Biological Process: ceramide metabolic process; cholesterol metabolic process; nervous system development; phospholipid metabolic process Disease: Ceroid Lipofuscinosis, Neuronal, 8; Ceroid Lipofuscinosis, Neuronal, 8, Northern Epilepsy Variant |
NCBI Summary: | This gene encodes a transmembrane protein belonging to a family of proteins containing TLC domains, which are postulated to function in lipid synthesis, transport, or sensing. The protein localizes to the endoplasmic reticulum (ER), and may recycle between the ER and ER-Golgi intermediate compartment. Mutations in this gene are associated with progressive epilepsy with mental retardation (EMPR), which is a subtype of neuronal ceroid lipofuscinoses (NCL). Patients with mutations in this gene have altered levels of sphingolipid and phospholipids in the brain. [provided by RefSeq, Jul 2008] |
UniProt Code: | Q9UBY8 |
NCBI GenInfo Identifier: | 145559455 |
NCBI Gene ID: | 2055 |
NCBI Accession: | Q9UBY8.3 |
UniProt Secondary Accession: | Q9UBY8,Q86U71, Q96I95, |
UniProt Related Accession: | Q9UBY8 |
Molecular Weight: | 32,787 Da |
NCBI Full Name: | Protein CLN8 |
NCBI Synonym Full Names: | ceroid-lipofuscinosis, neuronal 8 |
NCBI Official Symbol: | CLN8 |
NCBI Official Synonym Symbols: | EPMR; C8orf61 |
NCBI Protein Information: | protein CLN8 |
UniProt Protein Name: | Protein CLN8 |
Protein Family: | Protein |
UniProt Gene Name: | CLN8 |
UniProt Entry Name: | CLN8_HUMAN |
Antibodies |
Anti-CLN8 Antibody (CAB16843) |
Secondary Antibody |
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