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Bioss
Numéro de catalogue:
(BOSSBS-13333R-A488)
Fournisseur:
Bioss
Description:
Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of motor neurons in the spinal cord. SMA is caused by deletion or loss-of-function mutations in the SMN (Survival of Motor Neuron) gene. Gemin6, the protein product of human chromosome 2p22.2, associates directly with SMN and is a part of the SMN complex containing Gemin2, Gemin3, Gemin4 and Gemin5 as well as several spliceosomal snRNP proteins. The SMN complex plays an essential role in spliceosomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus. The SMN complex is found in both the cytoplasm and the nucleus. The nuclear form is concentrated in subnuclear bodies called gems (Gemini of the coiled bodies).
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-0860R-A488)
Fournisseur:
Bioss
Description:
HHV8 has been found to be associated with three different diseases observed in AIDS patients; kaposi's sarcoma, primary effusion lymphoma (which is a rare type of non-Hodgkin lymphoma affecting the body cavities) and multicentric Castleman's disease. To date there is much evidence to support a direct role for HHV8 in kaposi's sarcoma.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-2511R-A350)
Fournisseur:
Bioss
Description:
Terminates signal transduction at the neuromuscular junction by rapid hydrolysis of the acetylcholine released into the synaptic cleft. Role in neuronal apoptosis.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-13333R-A555)
Fournisseur:
Bioss
Description:
Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of motor neurons in the spinal cord. SMA is caused by deletion or loss-of-function mutations in the SMN (Survival of Motor Neuron) gene. Gemin6, the protein product of human chromosome 2p22.2, associates directly with SMN and is a part of the SMN complex containing Gemin2, Gemin3, Gemin4 and Gemin5 as well as several spliceosomal snRNP proteins. The SMN complex plays an essential role in spliceosomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus. The SMN complex is found in both the cytoplasm and the nucleus. The nuclear form is concentrated in subnuclear bodies called gems (Gemini of the coiled bodies).
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-1665R-CY3)
Fournisseur:
Bioss
Description:
Growth factor active in angiogenesis, vasculogenesis and endothelial cell growth. Induces endothelial cell proliferation, promotes cell migration, inhibits apoptosis and induces permeabilization of blood vessels. Binds to the FLT1/VEGFR1 and KDR/VEGFR2 receptors, heparan sulfate and heparin. NRP1/Neuropilin-1 binds isoforms VEGF-165 and VEGF-145. Isoform VEGF165B binds to KDR but does not activate downstream signaling pathways, does not activate angiogenesis and inhibits tumor growth.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-3836R-CY7)
Fournisseur:
Bioss
Description:
Binds the poly(A) tail of mRNA, including that of its own transcript. May be involved in cytoplasmic regulatory processes of mRNA metabolism such as pre-mRNA splicing. Its function in translational initiation regulation can either be enhanced by PAIP1 or repressed by PAIP2. Can probably bind to cytoplasmic RNA sequences other than poly(A) in vivo. Involved in translationally coupled mRNA turnover. Implicated with other RNA-binding proteins in the cytoplasmic deadenylation/translational and decay interplay of the FOS mRNA mediated by the major coding-region determinant of instability (mCRD) domain. Involved in regulation of nonsense-mediated decay (NMD) of mRNAs containing premature stop codons; for the recognition of premature termination codons (PTC) and initiation of NMD a competitive interaction between UPF1 and PABPC1 with the ribosome-bound release factors is proposed.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-3836R-A350)
Fournisseur:
Bioss
Description:
Binds the poly(A) tail of mRNA, including that of its own transcript. May be involved in cytoplasmic regulatory processes of mRNA metabolism such as pre-mRNA splicing. Its function in translational initiation regulation can either be enhanced by PAIP1 or repressed by PAIP2. Can probably bind to cytoplasmic RNA sequences other than poly(A) in vivo. Involved in translationally coupled mRNA turnover. Implicated with other RNA-binding proteins in the cytoplasmic deadenylation/translational and decay interplay of the FOS mRNA mediated by the major coding-region determinant of instability (mCRD) domain. Involved in regulation of nonsense-mediated decay (NMD) of mRNAs containing premature stop codons; for the recognition of premature termination codons (PTC) and initiation of NMD a competitive interaction between UPF1 and PABPC1 with the ribosome-bound release factors is proposed.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-11339R-A647)
Fournisseur:
Bioss
Description:
Members of the calsyntenin protein family are localized to the post-synaptic membrane of exicitatory central nervous system (CNS) synapses. Calsyntenin-2, also known as Alcadein-gamma, is a 955 amino acid protein that localizes to the endoplasmic reticulum, golgi apparatus and plasma membranes. Containing 2 cadherin-like repeats in its N-terminal extracellular region, calsyntenin-2 binds synaptic calcium with its cytoplasmic domain, suggesting a role in the modulation of calcium-mediated postsynaptic signals. Under normal physiological conditions, calsyntenin-2 is protoeolytically processed in an event in which the primary zeta-cleavage generates a short C-terminal transmembrane fragment and a long extracellular N-terminal domain.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-9166R-HRP)
Fournisseur:
Bioss
Description:
The protein encoded by this gene is a member of the tripartite motif (TRIM) family. The TRIM motif includes three zinc binding domains, a RING, a B box type 1 and a B box type 2, and a coiled coil region. The protein localizes to cytoplasmic bodies. Its function has not been identified. Alternative splicing of this gene generates three transcript variants, named alpha, beta and gamma.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-9018R-FITC)
Fournisseur:
Bioss
Description:
Chromosome 7 is about 158 milllion bases long, encodes over 1000 genes and makes up about 5% of the human genome. Chromosome 7 has been linked to Osteogenesis imperfecta, Pendred syndrome, Lissencephaly, Citrullinemia and Shwachman-Diamond syndrome. The deletion of a portion of the q arm of chromosome 7 is associated with Williams-Beuren syndrome, a condition characterized by mild mental retardation, an unusual comfort and friendliness with strangers and an elfin appearance. Deletions of portions of the q arm of chromosome 7 are also seen in a number of myeloid disorders including cases of acute myelogenous leukemia and myelodysplasia.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-3254R-HRP)
Fournisseur:
Bioss
Description:
Nuclear lamins form a network of intermediate-type filaments at the nucleoplasmic site of the nuclear membrane. Two main subtypes of nuclear lamins can be distinguished, i.e. A type lamins and B type lamins. The A type lamins comprise a set of three proteins arising from the same gene by alternative splicing, i.e. lamin A, lamin C and lamin Adel 10, while the B type lamins include two proteins arising from two distinct genes, i.e. lamin B1 and lamin B2. Recent evidence has revealed that mutations in A-type lamins give rise to a range of rare but dominant genetic disorders, including Emery-Dreifuss muscular dystrophy, dilated cardiomyopathy with conduction-system disease and Dunnigan-type familial partial lipodystrophy. In addition, the expression of A type lamins coincides with cell differentiation and as A type lamins specifically interact with chromatin, a role in the regulation of differential gene expression has been suggested for A type lamins.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-10341R-FITC)
Fournisseur:
Bioss
Description:
This gene encodes the K-type mitochondrial glutaminase. The encoded protein is an phosphate-activated amidohydrolase that catalyzes the hydrolysis of glutamine to glutamate and ammonia. This protein is primarily expressed in the brain and kidney plays an essential role in generating energy for metabolism, synthesizing the brain neurotransmitter glutamate and maintaining acid-base balance in the kidney. Alternate splicing results in multiple transcript variants. [provided by RefSeq, Jan 2012].
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-6796R-A555)
Fournisseur:
Bioss
Description:
Binds to lipid droplets and regulates their enlargement, thereby restricting lipolysis and favoring storage. At focal contact sites between lipid droplets, promotes directional net neutral lipid transfer from the smaller to larger lipid droplets. The transfer direction may be driven by the internal pressure difference between the contacting lipid droplet pair. Its role in neutral lipid transfer and lipid droplet enlargement is activated by the interaction with PLIN1. May act as a CEBPB coactivator in the white adipose tissue to control the expression of a subset of CEBPB downstream target genes, including SOCS1, SOCS3, TGFB1, TGFBR1, ID2 and XDH. When overexpressed in preadipocytes, induces apoptosis or increases cell susceptibility to apoptosis induced by serum deprivation or TGFB treatment. As mature adipocytes, that express high CIDEC levels, are quite resistant to apoptotic stimuli, the physiological significance of its role in apoptosis is unclear. May play a role in the modulation of the response to osmotic stress by preventing NFAT5 to translocate into the nucleus and activate its target genes expression.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-3254R-CY7)
Fournisseur:
Bioss
Description:
Nuclear lamins form a network of intermediate-type filaments at the nucleoplasmic site of the nuclear membrane. Two main subtypes of nuclear lamins can be distinguished, i.e. A type lamins and B type lamins. The A type lamins comprise a set of three proteins arising from the same gene by alternative splicing, i.e. lamin A, lamin C and lamin Adel 10, while the B type lamins include two proteins arising from two distinct genes, i.e. lamin B1 and lamin B2. Recent evidence has revealed that mutations in A-type lamins give rise to a range of rare but dominant genetic disorders, including Emery-Dreifuss muscular dystrophy, dilated cardiomyopathy with conduction-system disease and Dunnigan-type familial partial lipodystrophy. In addition, the expression of A type lamins coincides with cell differentiation and as A type lamins specifically interact with chromatin, a role in the regulation of differential gene expression has been suggested for A type lamins.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-5216R-A647)
Fournisseur:
Bioss
Description:
Bad is a member of the Bcl2 family and acts to promote apoptosis by forming heterodimers with the survival proteins Bcl2 and BclxL, thus preventing them from binding with BAX. Bad is found on the outer mitochondrial membrane and, once phosphorylated in response to growth stimuli, translocates to the cytoplasm. The phosphorylation status of Bad represents a key checkpoint for death or cell survival. JNK-induced phosphorylation of BAD serine 128 promotes the apoptotic role of Bad by opposing the inhibitory effect of growth factor on Bad-mediated apoptosis. Cdc2-induced phosphorylation of Bad serine 128 has an inhibitory effect on its interaction with 14-3-3 proteins. The latter interaction is critical for Bad phosphorylation at serine 155, a site within the BH3 domain that leads to the release of BclxL and the promotion of cell survival. Alternative splicing of this gene results in two transcript variants which encode the same isoform.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-5215R-A750)
Fournisseur:
Bioss
Description:
Cerebral deposition of amyloid beta peptide is an early and critical feature of Alzheimer's disease. Amyloid beta peptide is generated by proteolytic cleavage of amyloid precursor protein(APP) by two proteases, one of which is the protein encoded by this gene. The encoded protein, a member of the peptidase A1 protein family, is a type I integral membrane glycoprotein and aspartic protease that is found mainly in the Golgi. Multiple transcript variants encoding different isoforms have been described for this gene.
UOM:
1 * 100 µl
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