SEC63 Antibody

Code CSB-PA020961GA01HU
Size $600
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Product Details

Uniprot No.
Target Names
Alternative Names
SEC63; SEC63L; Translocation protein SEC63 homolog
Raised in
Rabbit
Species Reactivity
Human,Mouse,Rat
Immunogen
Human SEC63
Immunogen Species
Homo sapiens (Human)
Isotype
IgG
Purification Method
Antigen Affinity purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
Tested Applications
ELISA,WB
Troubleshooting and 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.

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Target Background

Function
Mediates cotranslational and post-translational transport of certain precursor polypeptides across endoplasmic reticulum (ER). Proposed to play an auxiliary role in recognition of precursors with short and apolar signal peptides. May cooperate with SEC62 and HSPA5/BiP to facilitate targeting of small presecretory proteins into the SEC61 channel-forming translocon complex, triggering channel opening for polypeptide translocation to the ER lumen. Required for efficient PKD1/Polycystin-1 biogenesis and trafficking to the plasma membrane of the primary cilia.
Gene References into Functions
  1. Results suggest that loss of PRKCSH and SEC63 leads to general defects in ciliogenesis, while quenching of the Wnt signaling cascade is cholangiocyte-restricted. PMID: 28973524
  2. a SEC63 germline mutation may play a role in cyst formation in polycystic liver disease PMID: 23209713
  3. Phosphorylation of Sec63 by CK2 enhanced its binding to Sec62 PMID: 23287549
  4. Sec63 may perform a substrate-selective quantity control function during cotranslational endoplasmic reticulum import. PMID: 23166619
  5. Silencing the human SEC63 genes inhibits transport of only a subset of signal-peptide-containing precursor proteins to endoplasmic reticulum. PMID: 22375059
  6. identified nucleoredoxin as an interaction partner of Sec63; characterized this interaction; Sec63 is linked to the Wnt signaling pathways and this interaction may be the reason why mutations in SEC63 can lead to polycystic liver disease PMID: 21251912
  7. identified a total of 26 novel mutations in PRKCSH (n = 14) and SEC63 (n = 12), including four splice site mutations, eight insertions/ deletions, six non-sense mutations, and eight missense mutations PMID: 20095989
  8. Mutations in SEC63 cause autosomal dominant polycystic liver disease, suggesting a role for cotranslational protein-processing pathways in maintaining epithelial luminal structure and implicating noncilial ER proteins in human polycystic disease. PMID: 15133510
  9. Sec63p expression was observed in all cyst epithelia regardless of mutational state. And, Cystogenesis in SEC63-associated PCLD occurs via a different mechanism. PMID: 18224332

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Involvement in disease
Polycystic liver disease 2 (PCLD2)
Subcellular Location
Endoplasmic reticulum membrane; Multi-pass membrane protein.
Tissue Specificity
Widely expressed, with high levels in the liver.
Database Links

HGNC: 21082

OMIM: 608648

KEGG: hsa:11231

STRING: 9606.ENSP00000357998

UniGene: Hs.26904

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