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Bioss
Numéro de catalogue:
(BOSSBS-5509R-A555)
Fournisseur:
Bioss
Description:
This gene encodes a 105 kD protein which can undergo cotranslational processing by the 26S proteasome to produce a 50 kD protein. The 105 kD protein is a Rel protein-specific transcription inhibitor and the 50 kD protein is a DNA binding subunit of the NF-kappa-B (NFKB) protein complex. NFKB is a transcription regulator that is activated by various intra- and extra-cellular stimuli such as cytokines, oxidant-free radicals, ultraviolet irradiation, and bacterial or viral products. Activated NFKB translocates into the nucleus and stimulates the expression of genes involved in a wide variety of biological functions. Inappropriate activation of NFKB has been associated with a number of inflammatory diseases while persistent inhibition of NFKB leads to inappropriate immune cell development or delayed cell growth. SUBUNIT: Active NF-kappa-B is a heterodimer of an about 50 kDa DNA-binding subunit and the weak DNA-binding subunit p65. Two heterodimers might form a labile tetramer. Also interacts with MAP3K8. NF-kappa-B p50 subunit interacts with NCOA3 coactivator, which may coactivate NF-kappa-B dependent expression via its histone acetyltransferase activity. Interacts with DSIPI; this interaction prevents nuclear translocation and DNA-binding. Interacts with SPAG9 and UNC5CL.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-13049R-A350)
Fournisseur:
Bioss
Description:
ECHDC2 is a 292 amino acid mitochondrial protein that exists as two isoforms which are produced by alternative splicing events. The gene encoding ECHDC2 maps to chromosome 1, which spans 260 million base pairs, contains over 3,000 genes and comprises nearly 8% of the human genome. Chromosome 1 encodes a large number of disease-associated proteins, including Lamin A which, when expressed abnormally, can build up in the nucleus and cause nuclear blebs, a characteristic of the rare aging disease Hutchinson-Gilford progeria. Additionally, genes that are involved in familial adenomatous polyposis, Stickler syndrome, Parkinsons Disease, Gaucher disease, schizophrenia and Usher syndrome are all located on chromosome 1. Aberrations in chromosome 1 are found in a variety of cancers, including head and neck cancer, malignant melanoma and multiple myeloma.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-9561R-A350)
Fournisseur:
Bioss
Description:
Penicillin refers to any member of beta-lactam antibiotics group. These agents are identified by a beta-lactam ring within their molecular structure. As the most widely used group of antibiotics available, beta-lactams are used for the treatment of bacterial infections usually caused by gram-positive organisms. Beta-lactam antibiotics are bactericidal, functioning to inhibit the synthesis of the peptidoglycan layer of bacterial cell walls. Bacterial penicillin-binding proteins and beta-lactamases constitute a large family of serine proteases that perform essential functions in the synthesis and maintenance of peptidoglycan cell wall. Notably, beta-lactamases cleave beta-lactams, therefore providing the bacteria with resistance to the antibiotic. Homologues of beta-lactamases occur in many species, including human, rat, cow, rabbit, pig, xenopus, zebrafish, and C. elegans. The human homologues, LACTB and LACTB2, are active-site-serine enzymes thought to be involved in metabolism.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-9500R-CY7)
Fournisseur:
Bioss
Description:
Hemostasis following tissue injury involves the deployment of essential plasma procoagulants (prothrombin, and factors X, IX, V, and VIII), which are involved in a blood coagulation cascade that leads to the formation of insoluble fibrin clots and the promotion of platelet aggregation (1-3). Coagulation factor IX (plasma thromboplastic component, F9, F.IX, HEMB) is a vitamin K-dependent, single chain serine protease that is synthesized in the liver and circulates as an inactive precursor (3,4). Factor XIa mediated proteolytic cleavage of factor IX generates factor IXa, an active serine protease composed of a 145 amino acid light chain and a 236 amino acid catalytic heavy chain, linked through disulfide bonds (5). Genetic alterations at the Factor IX locus such as point mutations, insertions and deletions, can lead to hemophilia B, also known as Christmas disease (6).
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-9500R-A350)
Fournisseur:
Bioss
Description:
Hemostasis following tissue injury involves the deployment of essential plasma procoagulants (prothrombin, and factors X, IX, V, and VIII), which are involved in a blood coagulation cascade that leads to the formation of insoluble fibrin clots and the promotion of platelet aggregation (1-3). Coagulation factor IX (plasma thromboplastic component, F9, F.IX, HEMB) is a vitamin K-dependent, single chain serine protease that is synthesized in the liver and circulates as an inactive precursor (3,4). Factor XIa mediated proteolytic cleavage of factor IX generates factor IXa, an active serine protease composed of a 145 amino acid light chain and a 236 amino acid catalytic heavy chain, linked through disulfide bonds (5). Genetic alterations at the Factor IX locus such as point mutations, insertions and deletions, can lead to hemophilia B, also known as Christmas disease (6).
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-9560R-A680)
Fournisseur:
Bioss
Description:
KRCC1 is a 259 amino acid protein that is encoded by a gene located on human chromosome 2p11.2. Consisting of 237 million bases, chromosome 2 is the second largest human chromosome and encodes over 1400 genes. A number of genetic diseases are linked to genes on chromosome 2. Harlequin icthyosis, a rare and morbid skin deformity, is associated with mutations in the ABCA12 gene. The lipid metabolic disorder sitosterolemia is associated with ABCG5 and ABCG8. An extremely rare recessive genetic disorder, Alstr syndrome, is due to mutations in the ALMS1 gene. Interestingly, chromosome 2 contains what appears to be a vestigial second centromere and vestigial telomeres which gives credence to the hypothesis that human chromosome 2 is the result of an ancient fusion of two ancestral chromosomes seen in modern form today in apes.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-9499R-A750)
Fournisseur:
Bioss
Description:
Making up nearly 6% of the human genome, chromosome 6 contains around 1200 genes within 170 million base pairs of sequence. Deletion of a portion of the q arm of chromosome 6 is associated with early onset intestinal cancer suggesting the presence of a cancer susceptibility locus. Porphyria cutanea tarda is associated with chromosome 6 through the HFE gene which, when mutated, predisposes an individual to developing this porphyria. Notably, the PARK2 gene, which is associated with Parkinson's disease, and the genes encoding the major histocompatibility complex proteins, which are key molecular components of the immune system and determine predisposition to rheumatic diseases, are also located on chromosome 6. Stickler syndrome, 21-hydroxylase deficiency and maple syrup urine disease are also associated with genes on chromosome 6. A bipolar disorder susceptibility locus has been identified on the q arm of chromosome 6. The C6orf62 gene product has been provisionally designated C6orf62 pending further characterisation.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-12566R-A680)
Fournisseur:
Bioss
Description:
ACAA1 is a 424 amino acid member of the thiolase family of enzymes and is involved in lipid metabolism. localised to the peroxisome, ACAA1 catalyses the conversion of acyl-CoA and acetyl-CoA to 3-oxoacyl-CoA in the fatty acid oxidation pathway. ACAA1 shows high enzymatic activity in liver, kidney, intestine and white adipose tissue in rats, where it exists as two types, namely type A and type B. Human ACAA1 shares 86% amino acid identity with its rat counterpart, suggesting a conserved function for ACAA1 among different species.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-12159R-A555)
Fournisseur:
Bioss
Description:
May act as a calcium-activated chloride channel.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-9500R-A555)
Fournisseur:
Bioss
Description:
Hemostasis following tissue injury involves the deployment of essential plasma procoagulants (prothrombin, and factors X, IX, V, and VIII), which are involved in a blood coagulation cascade that leads to the formation of insoluble fibrin clots and the promotion of platelet aggregation (1-3). Coagulation factor IX (plasma thromboplastic component, F9, F.IX, HEMB) is a vitamin K-dependent, single chain serine protease that is synthesized in the liver and circulates as an inactive precursor (3,4). Factor XIa mediated proteolytic cleavage of factor IX generates factor IXa, an active serine protease composed of a 145 amino acid light chain and a 236 amino acid catalytic heavy chain, linked through disulfide bonds (5). Genetic alterations at the Factor IX locus such as point mutations, insertions and deletions, can lead to hemophilia B, also known as Christmas disease (6).
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-12313R-HRP)
Fournisseur:
Bioss
Description:
The PHEX a 749 amino acid protein that putatively consists of an intracellular, transmembrane and extracellular domain. PHEX mutations have been observed in 60-80% of hypophosphatemic rickets patients. The PHEX protein, which is a single-pass membrane protein, is also designated HYP, X-linked hypophosphatemia protein or metalloendopeptidase homolog PEX. PHEX plays an active role in bone and dentin mineralization and renal phosphate re-absorption. X-linked hypophosphatemic rickets, also designated HYP, is an X-linked dominant disorder characterized by impaired phosphate uptake in the kidney, which is likely to be caused by abnormal regulation of sodium phosphate cotransport in the proximal tubules. Clinical manifestations include skeletal deformities, growth failure, craniosynostosis, paravertebral calcifications, pseudofractures in lower extremities, and muscular hypotonia with onset in early childhood.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-0161R-CY3)
Fournisseur:
Bioss
Description:
Plays a role in the regulation of the translocation of GLUT4 to the cell surface in adipocytes and skeletal muscle cells in response to insulin, probably by regulating RAB31 activity, and thereby contributes to the regulation of insulin-dependent glucose uptake. Low affinity receptor which can bind to NGF, BDNF, NT-3, and NT-4. Can mediate cell survival as well as cell death of neural cells. Necessary for the circadian oscillation of the clock genes ARNTL/BMAL1, PER1, PER2 and NR1D1 in the suprachiasmatic nucleus (SCN) of the brain and in liver and of the genes involved in glucose and lipid metabolism in the liver.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-13312R-A680)
Fournisseur:
Bioss
Description:
GCDH is a 438 amino acid protein that localizes to the mitochondrial matrix and belongs to the acyl-CoA dehydrogenase family. Existing as a homotetramer, GCDH uses FAD as a cofactor to catalyze the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine and L-tryptophan metabolism. While GCDH exists as both a long and short isoform, only the long isoform is a functionally active protein. Defects in the gene encoding GCDH are the cause of glutaric acidemia type I (GA-I), an autosomal recessive disorder that is characterized by the accumulation of glutaconic acid and is associated with such symptoms as progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-6162R-A647)
Fournisseur:
Bioss
Description:
Small GTP-binding protein which cycles between an inactive GDP-bound and an active GTP-bound form, and the rate of cycling is regulated by guanine nucleotide exchange factors (GEF) and GTPase-activating proteins (GAP). GTP-binding protein that does not act as an allosteric activator of the cholera toxin catalytic subunit. Requires assistance from GTPase-activating proteins (GAPs) like RP2 and PDE6D, in order to cycle between inactive GDP-bound and active GTP-bound forms. Required for normal cytokinesis and cilia signaling.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-15053R-A488)
Fournisseur:
Bioss
Description:
C1orf195
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-3357R-A350)
Fournisseur:
Bioss
Description:
Non-receptor protein-tyrosine kinase that regulates reorganization of the actin cytoskeleton, cell polarization, cell migration, adhesion, spreading and bone remodeling. Plays a role in the regulation of the humoral immune response, and is required for normal levels of marginal B-cells in the spleen and normal migration of splenic B-cells. Required for normal macrophage polarization and migration towards sites of inflammation. Regulates cytoskeleton rearrangement and cell spreading in T-cells, and contributes to the regulation of T-cell responses. Promotes osteoclastic bone resorption; this requires both PTK2B/PYK2 and SRC. May inhibit differentiation and activity of osteoprogenitor cells. Functions in signaling downstream of integrin and collagen receptors, immune receptors, G-protein coupled receptors (GPCR), cytokine, chemokine and growth factor receptors, and mediates responses to cellular stress. Forms multisubunit signaling complexes with SRC and SRC family members upon activation; this leads to the phosphorylation of additional tyrosine residues, creating binding sites for scaffold proteins, effectors and substrates. Regulates numerous signaling pathways. Promotes activation of phosphatidylinositol 3-kinase and of the AKT1 signaling cascade. Regulates production of the cellular messenger cGMP. Promotes activation of the MAP kinase signaling cascade, including activation of MAPK1/ERK2, MAPK3/ERK1 and MAPK8/JNK1. Promotes activation of Rho family GTPases, such as RHOA and RAC1. Recruits the ubiquitin ligase MDM2 to P53/TP53 in the nucleus, and thereby regulates P53/TP53 activity, P53/TP53 ubiquitination and proteasomal degradation. Acts as a scaffold, binding to both PDPK1 and SRC, thereby allowing SRC to phosphorylate PDPK1 at 'Tyr-9, 'Tyr-373', and 'Tyr-376'. Promotes phosphorylation of NMDA receptors by SRC family members, and thereby contributes to the regulation of NMDA receptor ion channel activity and intracellular Ca(2+) levels.
UOM:
1 * 100 µl
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