6-Fluorobenzo[b]thiophene-2-carboxylic+acid
Catalog Number:
(10295-012)
Supplier:
Bioss
Description:
Glucose-6-phosphatase (G6Pase), is a multicomponent enzyme system that hydrolyzes glucose-6-phosphate (G6P) in the final step of gluconeogenesis and gluconeolysis. G6Pase localizes to the endoplasmic reticulum, and while liver, kidney, and intestine are the only tissues that express the first identified isoform, G6Pase-Alpha, a second form, designated G6Pase-Beta, contributes to blood glucose homeostasis in a wider range of tissues. G6Pase-Beta, also known as SCN4, UGRP or G6PC3 (glucose 6 phosphatase, catalytic, 3), is a 346 amino acid endoplasmic reticulum multi-pass membrane protein that is involved in carbohydrate biosynthesis and the gluconeogenesis pathway. Inhibited by vanadate, G6Pase-Beta hydrolyzes GP6 to glucose in the endoplasmic reticulum. Due to its necessary involvement in normal glucose metabolism, G6Pase-Beta may play an integral role in diabetes and glycogen storage diseases (GSDs).
Catalog Number:
(10297-222)
Supplier:
Bioss
Description:
The glycine cleavage system is comprised of AMT (known as Protein T), GCSH (known as Protein H), DLD (known as Protein L) and GLDC (known as Protein P), all of which work together to catalyze the cleavage and degradation of glycine. GLDC (glycine dehydrogenase ), also known as GCE, GCSP (glycine cleavage system P protein) or HYGN1, is a 1,020 amino acid protein that localizes to the mitochondria and belongs to the gcvP family. GLDC binds to glycine and enables the methylamine group from glycine to be transferred to the Protein T. GLDC exists as a homodimer and utilizes pyridoxal phosphate as a cofactor. Mutations in the gene encoding GLDC leads to nonketotic hyperglycinemia (NKH), also known as glycine encephalopathy (GCE), an autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms.
Catalog Number:
(10295-006)
Supplier:
Bioss
Description:
Glucose-6-phosphatase (G6Pase), is a multicomponent enzyme system that hydrolyzes glucose-6-phosphate (G6P) in the final step of gluconeogenesis and gluconeolysis. G6Pase localizes to the endoplasmic reticulum, and while liver, kidney, and intestine are the only tissues that express the first identified isoform, G6Pase-Alpha, a second form, designated G6Pase-Beta, contributes to blood glucose homeostasis in a wider range of tissues. G6Pase-Beta, also known as SCN4, UGRP or G6PC3 (glucose 6 phosphatase, catalytic, 3), is a 346 amino acid endoplasmic reticulum multi-pass membrane protein that is involved in carbohydrate biosynthesis and the gluconeogenesis pathway. Inhibited by vanadate, G6Pase-Beta hydrolyzes GP6 to glucose in the endoplasmic reticulum. Due to its necessary involvement in normal glucose metabolism, G6Pase-Beta may play an integral role in diabetes and glycogen storage diseases (GSDs).
Catalog Number:
(10301-202)
Supplier:
Bioss
Description:
G protein-coupled receptors (GPCRs), also designated seven transmembrane (7TM) receptors and heptahelical receptors, are a protein family which interact with G proteins (heterotrimeric GTPases) to synthesize intracellular second messengers such as diacylglycerol, cyclic AMP, inositol phosphates, and calcium ions. Their diverse biological functions range from vision and olfaction to neuronal and endocrine signaling and are involved in many pathological conditions. G protein receptor 54 (GPR54), a member of the rhodopsin family of GCPRs, is the receptor for the Kiss1 gene product, metastin. Mutations in GPCR54 are associated with a lack of puberty onset and autosomal recessive idiopathic hypogonadotropic hypogonadism, a deficient or decreased function of the gonads. Proper function of GRP54 is essential for puberty. In the rat, GRP54 is expressed in the liver, intestine and most areas of the brain, while in the human it is expressed in the placenta, pituitary, pancreas and spinal cord.
Catalog Number:
(75791-964)
Supplier:
Prosci
Description:
Platelet-derived growth factor receptors (PDGFR) are cell surface tyrosine kinase receptors for members of the platelet-derived growth factor (PDGF) family. The PDGF family consists of PDGF-A, -B, -C and -D, which form either homo- or heterodimers (PDGF-AA, -AB, -BB, -CC, -DD). The four PDGFs are inactive in their monomeric forms. PDGFs bind to the protein tyrosine kinase receptors PDGF receptor- alpha and - beta . These two receptor isoforms dimerize upon binding the PDGF dimer, leading to three possible receptor combinations, namely - alpha alpha, - beta beta and - alpha beta . PDGFR alpha and PDGFR beta are members of the class III RTK family. Inappropriate PDGFR alpha and PDGFR beta signaling has been linked to a number of proliferative disorders.
Catalog Number:
(10297-236)
Supplier:
Bioss
Description:
The glycine cleavage system is comprised of AMT (known as Protein T), GCSH (known as Protein H), DLD (known as Protein L) and GLDC (known as Protein P), all of which work together to catalyze the cleavage and degradation of glycine. GLDC (glycine dehydrogenase ), also known as GCE, GCSP (glycine cleavage system P protein) or HYGN1, is a 1,020 amino acid protein that localizes to the mitochondria and belongs to the gcvP family. GLDC binds to glycine and enables the methylamine group from glycine to be transferred to the Protein T. GLDC exists as a homodimer and utilizes pyridoxal phosphate as a cofactor. Mutations in the gene encoding GLDC leads to nonketotic hyperglycinemia (NKH), also known as glycine encephalopathy (GCE), an autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms.
Catalog Number:
(10295-008)
Supplier:
Bioss
Description:
Glucose-6-phosphatase (G6Pase), is a multicomponent enzyme system that hydrolyzes glucose-6-phosphate (G6P) in the final step of gluconeogenesis and gluconeolysis. G6Pase localizes to the endoplasmic reticulum, and while liver, kidney, and intestine are the only tissues that express the first identified isoform, G6Pase-Alpha, a second form, designated G6Pase-Beta, contributes to blood glucose homeostasis in a wider range of tissues. G6Pase-Beta, also known as SCN4, UGRP or G6PC3 (glucose 6 phosphatase, catalytic, 3), is a 346 amino acid endoplasmic reticulum multi-pass membrane protein that is involved in carbohydrate biosynthesis and the gluconeogenesis pathway. Inhibited by vanadate, G6Pase-Beta hydrolyzes GP6 to glucose in the endoplasmic reticulum. Due to its necessary involvement in normal glucose metabolism, G6Pase-Beta may play an integral role in diabetes and glycogen storage diseases (GSDs).
Catalog Number:
(10260-006)
Supplier:
Bioss
Description:
Myotubularin and the myotubularin-related proteins (MTMR1-9) belong to a highly conserved family of eukaryotic phosphatases. They are protein tyrosine phosphatases that utilize inositol phospholipids, rather than phosphoproteins, as substrates. MTMR family members hydrolyze both Phosphatidylinositol 3-phosphate (PtdIns3P) and PtdIns(3,5)P2. MTMR2 interacts with MTMR5, an inactive family member that increases the enzymatic activity of MTMR2 and dictates its subcellular localization. Mutations in MTMR2 cause autosomal recessive Charcot-Marie-Tooth type 4B1 (CMT4B1), which is characterized by reduced nerve conduction velocities, focally folded myelin sheaths and demyelination. MTMR3 and MTMR4 can either interact with each other or self associate. MTMR6 regulates the activity of the calcium-activated potassium channel 3.1. MTMR9 regulates the activity of MTMR7 and MTMR8.
Catalog Number:
(10252-870)
Supplier:
Bioss
Description:
This gene encodes the acidic form of complement factor 4, part of the classical activation pathway. The protein is expressed as a single chain precursor which is proteolytically cleaved into a trimer of alpha, beta, and gamma chains prior to secretion. The trimer provides a surface for interaction between the antigen-antibody complex and other complement components. The alpha chain may be cleaved to release C4 anaphylatoxin, a mediator of local inflammation. Deficiency of this protein is associated with systemic lupus erythematosus and type I diabetes mellitus. This gene localizes to the major histocompatibility complex (MHC) class III region on chromosome 6. Varying haplotypes of this gene cluster exist, such that individuals may have 1, 2, or 3 copies of this gene. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Nov 2011].
Catalog Number:
(10247-048)
Supplier:
Bioss
Description:
MYBPC1 is a 1,141 amino acid protein that contains three fibronectin type-III domains and seven Ig-like C2-type domains. Existing as a member of the immunoglobulin superfamily, MYBPC1 functions as a thick filament-associated protein that localizes to striated muscle bands in vertebrae and is thought to modify the activity of select ATPases. Additionally, MYBPC1 may play a role in the modulation of muscle contraction and in the overall structural integrity of the cell. The gene encoding MYBPC1 maps to human chromosome 12, which encodes over 1,100 genes and comprises approximately 4.5% of the human genome. Chromosome 12 is associated with a variety of diseases and afflictions, including hypochondrogenesis, achondrogenesis, Kniest dysplasia, Noonan syndrome and Trisomy 12p, which causes facial developmental defects and seizure disorders.
Catalog Number:
(10280-438)
Supplier:
Bioss
Description:
The apolipoprotein L gene family maps to a region on chromosome 22 and encodes six highly homologous proteins designated apoL-I, apoL-II, apoL-III, apoL-IV, apoL-V and apoL-VI, all of which function as components of plasma lipoproteins. ApoL-IV (apolipoprotein L-IV), also known as APOL4, is a 351 amino acid protein that exists as multiple alternatively spliced isoforms, one of which is secreted. Expressed in spleen, placenta, spinal cord, uterus, testis and trachea, apoL-IV is thought to play a role in lipid exchange and transport throughout the body and may be involved in reverse cholesterol transport, specifically from peripheral cells to the liver. Overexpression of apoL-IV is associated with schizophrenia, suggesting that apoL-IV may play a role in the pathogenesis of neural disorders.
Catalog Number:
(10268-694)
Supplier:
Bioss
Description:
Binds to DNA, at nuclear matrix- or scaffold-associated regions. Thought to recognize the sugar-phosphate structure of double-stranded DNA. Transcription factor controlling nuclear gene expression, by binding to matrix attachment regions (MARs) of DNA and inducing a local chromatin-loop remodeling. Acts as a docking site for several chromatin remodeling enzymes and also by recruiting corepressors (HDACs) or coactivators (HATs) directly to promoters and enhancers. Required for the initiation of the upper-layer neurons (UL1) specific genetic program and for the inactivation of deep-layer neurons (DL) and UL2 specific genes, probably by modulating BCL11B expression. Repressor of Ctip2 and regulatory determinant of corticocortical connections in the developing cerebral cortex. May play an important role in palate formation. Acts as a molecular node in a transcriptional network regulating skeletal development and osteoblast differentiation.
Catalog Number:
(10482-242)
Supplier:
Bioss
Description:
The pantothenate kinase (PANK) family of proteins catalyzes the first step in coenzyme A (CoA) biosynthesis. Coenzyme A is an important coenzyme involved in the synthesis and oxidation of fatty acids, as well as the oxidation of pyruvate in the citric acid (Krebs) cycle. Pantothenate kinase 3 (PANK3) is a 370 amino acid member of the pantothenate kinase family that plays a role in the physiological regulation of the intracellular CoA concentration. Localized to the cytoplasm, PANK3 is regulated by feedback inhibition by CoA and its thioesters. PANK3 transfers a phosphate from ATP to pantothenate (Vitamin B5), resulting in formation of 4’-phosphopantothenate. Closely related to its family members, PANK1, PANK2 and PANK4, PANK3 is highly expressed in liver. Pantothenate kinase associated neurodegeneration (PKAN) results from mutations in the gene encoding PANK2, the only mitochondria targeted human PANK.
Catalog Number:
(10482-260)
Supplier:
Bioss
Description:
The pantothenate kinase (PANK) family of proteins catalyzes the first step in coenzyme A (CoA) biosynthesis. Coenzyme A is an important coenzyme involved in the synthesis and oxidation of fatty acids, as well as the oxidation of pyruvate in the citric acid (Krebs) cycle. Pantothenate kinase 3 (PANK3) is a 370 amino acid member of the pantothenate kinase family that plays a role in the physiological regulation of the intracellular CoA concentration. Localized to the cytoplasm, PANK3 is regulated by feedback inhibition by CoA and its thioesters. PANK3 transfers a phosphate from ATP to pantothenate (Vitamin B5), resulting in formation of 4’-phosphopantothenate. Closely related to its family members, PANK1, PANK2 and PANK4, PANK3 is highly expressed in liver. Pantothenate kinase associated neurodegeneration (PKAN) results from mutations in the gene encoding PANK2, the only mitochondria targeted human PANK.
Catalog Number:
(10480-918)
Supplier:
Bioss
Description:
G protein-coupled receptors (GPCRs) represent a large superfamily of cell-surface receptors that are involved in a multitude of physiological processes such as perception of sensory information, modulation of synaptic transmission, hormone release/actions, regulation of cell contraction/migration and cell growth/differentiation. GPCRs interact with G proteins (heterotrimeric GTPases) to synthesize intracellular second messengers, such as diacylglycerol, cyclic AMP, inositol phosphates and calcium ions. Their diverse biological functions range from vision and olfaction to neuronal and endocrine signaling and are involved in many pathological conditions. GRIN2 (G protein-regulated inducer of neurite outgrowth 2), also known as GPRIN2, is a 458 amino acid protein that is expressed in cerebellum and is thought to play a role in neurite outgrowth. GRIN2 interacts with activated G?oand G?, and is encoded by a gene that maps to human chromosome 10q11.22.
Catalog Number:
(76108-584)
Supplier:
Bioss
Description:
The pantothenate kinase (PANK) family of proteins catalyzes the first step in coenzyme A (CoA) biosynthesis. Coenzyme A is an important coenzyme involved in the synthesis and oxidation of fatty acids, as well as the oxidation of pyruvate in the citric acid (Krebs) cycle. Pantothenate kinase 3 (PANK3) is a 370 amino acid member of the pantothenate kinase family that plays a role in the physiological regulation of the intracellular CoA concentration. Localized to the cytoplasm, PANK3 is regulated by feedback inhibition by CoA and its thioesters. PANK3 transfers a phosphate from ATP to pantothenate (Vitamin B5), resulting in formation of 4?-phosphopantothenate. Closely related to its family members, PANK1, PANK2 and PANK4, PANK3 is highly expressed in liver. Pantothenate kinase associated neurodegeneration (PKAN) results from mutations in the gene encoding PANK2, the only mitochondria targeted human PANK.
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