产品名称
Anti-SIL1 antibody produced in rabbit, affinity isolated antibody
biological source
rabbit
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
form
buffered aqueous solution
mol wt
49 kDa
species reactivity
human, guinea pig, bovine, rabbit, rat, horse
concentration
0.5 mg - 1 mg/mL
technique(s)
immunohistochemistry: suitable
western blot: suitable
NCBI accession no.
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Quality Level
Gene Information
human ... SIL1(64374)
Application
Anti-SIL1 antibody produced in rabbit is suitable for western blotting at a concentration of 0.25μg/ml and for immunohistochemistry of paraffin-embedded tissue sections at a concentration of 4-8μg/ml.
Biochem/physiol Actions
SIL1 nucleotide exchange factor is an N-linked glycoprotein present in the membrane of endoplasmic reticulum. In coordination with HSPA5, SIL1 regulates proper folding of newly synthesized of proteins and degrades proteins that fail to mature properly. SIL1 is also regulated by glucose and its activity is essential for glucose-stimulated insulin secretion by the pancreatic beta cells. Mutations in SIL1 cause cerebellar ataxia, chronic myopathy, cataracts, delayed motor development and intellectual disability, collectively known as Marinesco-Sjögren syndrome.
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Immunogen
Synthetic peptide directed towards the N terminal region of human SIL1
Other Notes
Synthetic peptide located within the following region: KETKAEEELDAEVLEVFHPTHEWQALQPGQAVPAGSHVRLNLQTGEREAK
Physical form
Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
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存储类别
10 - Combustible liquids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
法规信息
常规特殊物品
此项目有
Michael Krieger et al.
Brain : a journal of neurology, 136(Pt 12), 3634-3644 (2013-11-02)
Marinesco-Sjögren syndrome is a rare autosomal recessive multisystem disorder featuring cerebellar ataxia, early-onset cataracts, chronic myopathy, variable intellectual disability and delayed motor development. More recently, mutations in the SIL1 gene, which encodes an endoplasmic reticulum resident co-chaperone, were identified as
Yutaka Inaguma et al.
EMBO molecular medicine, 6(3), 414-429 (2014-01-30)
Marinesco-Sjögren syndrome (MSS) is a rare autosomal recessively inherited disorder with mental retardation (MR). Recently, mutations in the SIL1 gene, encoding a co-chaperone which regulates the chaperone HSPA5, were identified as a major cause of MSS. We here examined the
Arne A Ittner et al.
Diabetologia, 57(7), 1410-1419 (2014-04-16)
Regulation of insulin secretion along the secretory pathway is incompletely understood. We addressed the expression of SIL1, a nucleotide exchange factor for the endoplasmic reticulum (ER) chaperone glucose-regulated protein 78 kD (GRP78), in pancreatic beta cells and investigated whether or not
Kyung Tae Chung et al.
The Journal of biological chemistry, 277(49), 47557-47563 (2002-10-03)
We identified a mammalian BiP-associated protein, BAP, using a yeast two-hybrid screen that shared low homology with yeast Sls1p/Sil1p and mammalian HspBP1, both of which regulate the ATPase activity of their Hsp70 partner. BAP encoded an approximately 54-kDa protein with
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