CLN8 Antibody

Code CSB-PA866210LA01HU
Size US$299
Image
  • IHC image of CSB-PA866210LA01HU 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.

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Product Details

Full Product Name Rabbit anti-Homo sapiens (Human) CLN8 Polyclonal antibody
Uniprot No. Q9UBY8
Target Names CLN8
Alternative Names CLN8 antibody; C8orf61 antibody; Protein CLN8 antibody
Raised in Rabbit
Species Reactivity Human
Immunogen Recombinant Human Protein CLN8 protein (247-286AA)
Immunogen Species Homo sapiens (Human)
Conjugate Non-conjugated
Clonality Polyclonal
Isotype IgG
Purification Method >95%, Protein G purified
Concentration It differs from different batches. Please contact us to confirm it.
Buffer Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form Liquid
Tested Applications ELISA, IHC
Recommended Dilution
Application Recommended Dilution
IHC 1:20-1:200
Protocols ELISA Protocol
Immunohistochemistry (IHC) Protocol
Troubleshooting and FAQs Antibody FAQs
Storage Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Target Data

Function Could play a role in cell proliferation during neuronal differentiation and in protection against cell death.
Gene References into Functions
  1. CLN8 recruits lysosomal soluble proteins in the endoplasmic reticulum (ER), delivers them to the Golgi apparatus via COPII-coated vesicles, and recycles back to the ER via COPI-coated vesicles. CLN8 interacts with the lysosomal soluble proteins through its large luminal loop. The export signal of CLN8 (261VDWNF265) is localized in its cytosolic C-terminus. CLN8 deficiency results in depletion of enzymes at the lysosome. PMID: 30397314
  2. Whole-exome sequencing and homozygosity mapping revealed a novel homozygous CLN8 mutation, c.677T>C (p.Leu226Pro in 5 relatives from a large Turkish consanguineous family PMID: 27844444
  3. Novel missense mutation in CLN8 in late infantile neuronal ceroid lipofuscinosis PMID: 26443629
  4. This study does not support a contribution of rare missense CLN8 variations to ASD susceptibility in the Japanese population. PMID: 26657971
  5. This study highlights a close interaction between CLN5/CLN8 proteins, and their role in sphingolipid metabolism. Our findings suggest that CLN5p/CLN8p most likely are positive modulators of CerS1 and/or CerS2. PMID: 23160995
  6. A missense mutation at the CLN8 gene (763C>G)has been identified in 3 consanguineous Israeli-Arab patients. The phenotype in 2 of them is milder than that of their cousin who has typical neuronal ceroid lipofuscinosis. PMID: 22964447
  7. CLN8 is a candidate modifier gene for GD1. Increased expression may protect against severe GD1.It may function as a protective sphingolipid sensor and/or in glycosphingolipid trafficking. PMID: 22388998
  8. a novel, large CLN8 gene deletion c.544-2566_590del2613 in a Turkish family with a slightly more severe phenotype of neuronal ceroid lipofuscinose was described. PMID: 19807737
  9. patients with CLN8 mutations from Italy. In these patients, the onset of epilepsy occurred between 3 and 6 years of age, with myoclonic, tonic-clonic, and atypical absence seizures. Electroencephalograms revealed focal and/or generalized abnormalities. PMID: 17129765
  10. CLN8 plays a role in cell proliferation during neuronal differentiation and in protection against cell death. PMID: 19431184

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Involvement in disease Ceroid lipofuscinosis, neuronal, 8 (CLN8); Ceroid lipofuscinosis, neuronal, 8, Northern epilepsy variant (CLN8NE)
Subcellular Location Endoplasmic reticulum membrane, Multi-pass membrane protein, Endoplasmic reticulum-Golgi intermediate compartment membrane, Multi-pass membrane protein, Endoplasmic reticulum
Database Links

HGNC: 2079

OMIM: 600143

KEGG: hsa:2055

STRING: 9606.ENSP00000328182

UniGene: Hs.127675

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