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Artikel nummer : (BOSSBS-0136R-FITC)
Leverantör: Bioss
Beskrivning: GTP cyclohydrolase I (also designated dopa-responsive dystonia) catalyzes the conversion of GTP to D-erythro-7,8-dihydroneopterin triphosphate, the first and rate-limiting step in tetrahydrobiopterin (BH4) biosynthesis. Tetrahydrobiopterin is an essential cofactor for 3 aromatic amino acid monooxygenases: phenylalanine, tyrosine, and tryptophan hydroxylases. Animals can synthesize tetrahydrobiopterin in vivo from GTP through several enzymatic reactions.
UOM: 1 * 100 µl


Leverantör: MP Biomedicals
Beskrivning: D-Tryptophan is an amino acid and a unnatural isomer of tryptophan.

Artikel nummer : (BOSSBS-11489R-CY5)
Leverantör: Bioss
Beskrivning: TNRC6B is a 1,723 amino acid protein that exists as two alternatively spliced isoforms and is thought to be involved in mRNA cleavage events. Expressed ubiquitously, TNRC6B contains one glycine/tryptophan (GW)-rich N-terminal domain, one central glutamine-rich region and one C-terminal RNA recognition motif and is encoded by a gene that maps to human chromosome 22. Mutations in several of the genes that map to chromosome 22 are involved in the development of Phelan-McDermid syndrome, Neurofibromatosis type 2, autism and schizophrenia. Additionally, translocations between chromosomes 9 and 22 may lead to the formation of the Philadelphia Chromosome and the subsequent production of the novel fusion protein BCR-Abl, a potent cell proliferation activator found in several types of leukemias.
UOM: 1 * 100 µl


Artikel nummer : (BOSSBS-13312R-A350)
Leverantör: Bioss
Beskrivning: 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


Artikel nummer : (BOSSBS-13312R-CY7)
Leverantör: Bioss
Beskrivning: 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


Artikel nummer : (BOSSBS-13312R-A555)
Leverantör: Bioss
Beskrivning: 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


Artikel nummer : (BOSSBS-0121R-A488)
Leverantör: Bioss
Beskrivning: SYAP1 (synapse-associated protein 1) is a 352 amino acid protein that is ubiquitously expressed in adult tissues. SYAP1 contains one BSD domain which is a novel domain that is present in basal transcription factors, synapse-associated proteins and several hypothetical proteins. The BSD domain is characterized by three predicted alpha helices and by conserved tryptophan and phenylalanine residues, located at the C-terminus of the domain. The gene that encodes SYAP1 in humans is located on chromosome X. Chromosome X consists of about 153 million base pairs and nearly 1,000 genes. Color blindness, hemophilia, and Duchenne muscular dystrophy are well known X chromosome-linked conditions which affect males more frequently as males carry a single X chromosome.
UOM: 1 * 100 µl


Artikel nummer : (BOSSBS-11489R-A647)
Leverantör: Bioss
Beskrivning: TNRC6B is a 1,723 amino acid protein that exists as two alternatively spliced isoforms and is thought to be involved in mRNA cleavage events. Expressed ubiquitously, TNRC6B contains one glycine/tryptophan (GW)-rich N-terminal domain, one central glutamine-rich region and one C-terminal RNA recognition motif and is encoded by a gene that maps to human chromosome 22. Mutations in several of the genes that map to chromosome 22 are involved in the development of Phelan-McDermid syndrome, Neurofibromatosis type 2, autism and schizophrenia. Additionally, translocations between chromosomes 9 and 22 may lead to the formation of the Philadelphia Chromosome and the subsequent production of the novel fusion protein BCR-Abl, a potent cell proliferation activator found in several types of leukemias.
UOM: 1 * 100 µl


Artikel nummer : (BOSSBS-11784R-A647)
Leverantör: Bioss
Beskrivning: SPR, also known as sepiapterin reductase, is a homodimeric cytoplasmic protein that belongs to the sepiapterin reductase family. SPR functions as an NADH-dependent aldo-keto reductase and specifically catalyzes the reduction of pteridine derivatives. In addition, SPR plays an important role in tetrahydrobiopterin (BH4) biosynthesis, catalyzing the final reduction step of the synthesis pathway. BH4 is an essential cofactor for the hydroxylation of the aromatic amino acids (tryptophan, tyrosine and phenylalanine) and is required for proper dopamine synthesis. Mutations in the gene encoding SPR can cause sepiapterin reductase deficiency, a monoamine neurotransmitter deficiency without hyperphenylalaninemia. Sepiapterin reductase deficiency interferes with BH4 synthesis, resulting in DOPA-responsive dystonia and a variety of other human diseases. In addition, SPR mRNA expression is increased in the brain of Parkinson’s Disease (PD) patients, suggesting that SPR may play a role in PD.
UOM: 1 * 100 µl


Artikel nummer : (BOSSBS-11784R-A750)
Leverantör: Bioss
Beskrivning: SPR, also known as sepiapterin reductase, is a homodimeric cytoplasmic protein that belongs to the sepiapterin reductase family. SPR functions as an NADH-dependent aldo-keto reductase and specifically catalyses the reduction of pteridine derivatives. In addition, SPR plays an important role in tetrahydrobiopterin (BH4) biosynthesis, catalyzing the final reduction step of the synthesis pathway. BH4 is an essential cofactor for the hydroxylation of the aromatic amino acids (tryptophan, tyrosine and phenylalanine) and is required for proper dopamine synthesis. Mutations in the gene encoding SPR can cause sepiapterin reductase deficiency, a monoamine neurotransmitter deficiency without hyperphenylalaninemia. Sepiapterin reductase deficiency interferes with BH4 synthesis, resulting in DOPA-responsive dystonia and a variety of other human diseases. In addition, SPR mRNA expression is increased in the brain of Parkinson's Disease (PD) patients, suggesting that SPR may play a role in PD.
UOM: 1 * 100 µl


Artikel nummer : (BOSSBS-0121R-CY3)
Leverantör: Bioss
Beskrivning: SYAP1 (synapse-associated protein 1) is a 352 amino acid protein that is ubiquitously expressed in adult tissues. SYAP1 contains one BSD domain which is a novel domain that is present in basal transcription factors, synapse-associated proteins and several hypothetical proteins. The BSD domain is characterized by three predicted alpha helices and by conserved tryptophan and phenylalanine residues, located at the C-terminus of the domain. The gene that encodes SYAP1 in humans is located on chromosome X. Chromosome X consists of about 153 million base pairs and nearly 1,000 genes. Color blindness, hemophilia, and Duchenne muscular dystrophy are well known X chromosome-linked conditions which affect males more frequently as males carry a single X chromosome.
UOM: 1 * 100 µl


Artikel nummer : (BOSSBS-13312R-HRP)
Leverantör: Bioss
Beskrivning: 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


Artikel nummer : (BOSSBS-11489R-HRP)
Leverantör: Bioss
Beskrivning: TNRC6B is a 1,723 amino acid protein that exists as two alternatively spliced isoforms and is thought to be involved in mRNA cleavage events. Expressed ubiquitously, TNRC6B contains one glycine/tryptophan (GW)-rich N-terminal domain, one central glutamine-rich region and one C-terminal RNA recognition motif and is encoded by a gene that maps to human chromosome 22. Mutations in several of the genes that map to chromosome 22 are involved in the development of Phelan-McDermid syndrome, Neurofibromatosis type 2, autism and schizophrenia. Additionally, translocations between chromosomes 9 and 22 may lead to the formation of the Philadelphia Chromosome and the subsequent production of the novel fusion protein BCR-Abl, a potent cell proliferation activator found in several types of leukemias.
UOM: 1 * 100 µl


Artikel nummer : (BOSSBS-11489R-CY7)
Leverantör: Bioss
Beskrivning: TNRC6B is a 1,723 amino acid protein that exists as two alternatively spliced isoforms and is thought to be involved in mRNA cleavage events. Expressed ubiquitously, TNRC6B contains one glycine/tryptophan (GW)-rich N-terminal domain, one central glutamine-rich region and one C-terminal RNA recognition motif and is encoded by a gene that maps to human chromosome 22. Mutations in several of the genes that map to chromosome 22 are involved in the development of Phelan-McDermid syndrome, Neurofibromatosis type 2, autism and schizophrenia. Additionally, translocations between chromosomes 9 and 22 may lead to the formation of the Philadelphia Chromosome and the subsequent production of the novel fusion protein BCR-Abl, a potent cell proliferation activator found in several types of leukemias.
UOM: 1 * 100 µl


Artikel nummer : (BOSSBS-0121R-A647)
Leverantör: Bioss
Beskrivning: SYAP1 (synapse-associated protein 1) is a 352 amino acid protein that is ubiquitously expressed in adult tissues. SYAP1 contains one BSD domain which is a novel domain that is present in basal transcription factors, synapse-associated proteins and several hypothetical proteins. The BSD domain is characterized by three predicted alpha helices and by conserved tryptophan and phenylalanine residues, located at the C-terminus of the domain. The gene that encodes SYAP1 in humans is located on chromosome X. Chromosome X consists of about 153 million base pairs and nearly 1,000 genes. Color blindness, hemophilia, and Duchenne muscular dystrophy are well known X chromosome-linked conditions which affect males more frequently as males carry a single X chromosome.
UOM: 1 * 100 µl


Artikel nummer : (BOSSBS-11784R-CY7)
Leverantör: Bioss
Beskrivning: SPR, also known as sepiapterin reductase, is a homodimeric cytoplasmic protein that belongs to the sepiapterin reductase family. SPR functions as an NADH-dependent aldo-keto reductase and specifically catalyzes the reduction of pteridine derivatives. In addition, SPR plays an important role in tetrahydrobiopterin (BH4) biosynthesis, catalyzing the final reduction step of the synthesis pathway. BH4 is an essential cofactor for the hydroxylation of the aromatic amino acids (tryptophan, tyrosine and phenylalanine) and is required for proper dopamine synthesis. Mutations in the gene encoding SPR can cause sepiapterin reductase deficiency, a monoamine neurotransmitter deficiency without hyperphenylalaninemia. Sepiapterin reductase deficiency interferes with BH4 synthesis, resulting in DOPA-responsive dystonia and a variety of other human diseases. In addition, SPR mRNA expression is increased in the brain of Parkinson’s Disease (PD) patients, suggesting that SPR may play a role in PD.
UOM: 1 * 100 µl


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Lager för denna produkt är begränsat, men kan vara tillgänglig i ett lager nära dig. Se till att du är inloggad på hemsidan så att tillgängligt lager kan visas. Om call fortfarande visas och du behöver hjälp, ring oss på 08-621 34 20.
VWR International AB säljer bara etanol till skattebefriade kunder. Denna produkt ä reglerad enligt svensk lagstiftning. Om ytterligare information krävs av dig kommer du att kontaktas via e-post.
VWR International AB säljer bara etanol till skattebefriade kunder.
Denna produkt ä reglerad enligt svensk lagstiftning. Om ytterligare information krävs av dig kommer du att kontaktas via email.
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