Human SIL1 Recombinant Protein (RPPB4685)

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SKU:
RPPB4685
Product type:
Recombinant Protein
Size:
10ug
Species:
Human
Target:
SIL1
Synonyms:
SIL1 homolog endoplasmic reticulum chaperone (S cerevisiae)
Synonyms:
nucleotide exchange factor SIL1
Synonyms:
Marinesco-Sjogren syndrome
Synonyms:
BiP-associated protein
Source:
Escherichia Coli
Uniprot:
Q9H173
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Description

Product Name:Human SIL1 Recombinant Protein
Product Code:RPPB4685
Size:10µg
Species:Human
Target:SIL1
Synonyms:SIL1 homolog endoplasmic reticulum chaperone (S. cerevisiae), nucleotide exchange factor SIL1, Marinesco-Sjogren syndrome, BiP-associated protein, ULG5, MSS, BAP.
Source:Escherichia Coli
Physical Appearance:Sterile Filtered colorless solution.
Formulation:The SIL1 solution (0.5mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 100mM Nacl and 10% glycerol.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 90% as determined by SDS-PAGE.
Amino Acid Sequence:MHQNLKEFAL TNPEKSSTKE TERKETKAEE ELDAEVLEVF HPTHEWQALQ PGQAVPAGSH VRLNLQTGER EAKLQYEDKF RNNLKGKRLD INTNTYTSQD LKSALAKFKE GAEMESSKED KARQAEVKRL FRPIEELKKD FDELNVVIET DMQIMVRLIN KFNSSSSSLE EKIAALFDLE YYVHQMDNAQ DLLSFGGLQV VINGLNSTEP LVKEYAAFVL GAAFSSNPKV QVEAIEGGAL QKLLVILATE QPLTAKKKVL FALCSLLRHF PYAQRQFLKL GGLQVLRTLV QEKGTEVLAV RVVTLLYDLV TEKMFAEEEA ELTQEMSPEK LQQYRQVHLL PGLWEQGWCE ITAHLLALPE HDAREKVLQT LGVLLTTCRD RYRQDPQLGR TLASLQAEYQ VLASLELQDG EDEGYFQELL GSVNSLLKEL RLEHHHHHH

SIL1 is localized to endoplasmic reticulum (ER). SIL1 is an N-linked glycoprotein with an N-terminal ER targeting sequence, 2 putative N-glycosylation sites, and a C-terminal ER retention signal. SIL1 acts as a nucleotide exchange factor for another unfolded protein response protein. Mutations in SIL1 are linked to Marinesco-Sjogren syndrome.

SIL1 Human Recombinant produced in E. coli is a single polypeptide chain containing 439 amino acids (32-461) and having a molecular mass of 50.0 kDa.SIL1 is fused to a 8 amino acid His-tag at C-terminus & purified by proprietary chromatographic techniques.

UniProt Protein Function:SIL1: Required for protein translocation and folding in the endoplasmic reticulum (ER). Functions as a nucleotide exchange factor for the ER lumenal chaperone HSPA5. Defects in SIL1 are a cause of Marinesco-Sjoegren syndrome (MSS). MSS is an autosomal recessive multisystem disorder which is characterized by cerebellar ataxia due to cerebellar atrophy, with Purkinje and granule cell loss and myopathy featuring marked muscle replacement with fat and connective tissue. Other cardinal features include bilateral cataracts, hypergonadotrophic hypogonadism and mild to severe mental retardation. Skeletal abnormalities, short stature, dysarthria, strabismus and nystagmus are also frequent findings. Mutational inactivation of this protein may result in ER stress- induced cell death signaling or malfunctioning chaperone machineries that mishandle client proteins which are critical for the organs targeted in MSS. Belongs to the SIL1 family.Protein type: Secreted, signal peptide; SecretedChromosomal Location of Human Ortholog: 5q31Cellular Component: endoplasmic reticulum; extracellular spaceMolecular Function: protein bindingDisease: Marinesco-sjogren Syndrome
UniProt Protein Details:
NCBI Summary:This gene encodes a resident endoplasmic reticulum (ER), N-linked glycoprotein with an N-terminal ER targeting sequence, 2 putative N-glycosylation sites, and a C-terminal ER retention signal. This protein functions as a nucleotide exchange factor for another unfolded protein response protein. Mutations in this gene have been associated with Marinesco-Sjogren syndrome. Alternate transcriptional splice variants have been characterized. [provided by RefSeq, Jul 2008]
UniProt Code:Q9H173
NCBI GenInfo Identifier:74733533
NCBI Gene ID:64374
NCBI Accession:Q9H173.1
UniProt Secondary Accession:Q9H173,Q8N2L3, D3DQC2
UniProt Related Accession:Q9H173
Molecular Weight:52,085 Da
NCBI Full Name:Nucleotide exchange factor SIL1
NCBI Synonym Full Names:SIL1 nucleotide exchange factor
NCBI Official Symbol:SIL1  
NCBI Official Synonym Symbols:BAP; MSS; ULG5  
NCBI Protein Information:nucleotide exchange factor SIL1
UniProt Protein Name:Nucleotide exchange factor SIL1
UniProt Synonym Protein Names:BiP-associated protein; BAP
Protein Family:Silaffin
UniProt Gene Name:SIL1  
UniProt Entry Name:SIL1_HUMAN
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