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Artikel-Nr: (PRSI26-612)
Lieferant: ProSci Inc.
Hersteller Artikel Nummer : 26-612
Beschreibung: 4-aminobutyrate aminotransferase (ABAT) is responsible for catabolism of gamma-aminobutyric acid (GABA), an important, mostly inhibitory neurotransmitter in the central nervous system, into succinic semialdehyde. The active enzyme is a homodimer of 50-kD subunits complexed to pyridoxal-5-phosphate. ABAT in liver and brain is controlled by 2 codominant alleles with a frequency in a Caucasian population of 0.56 and 0.44. The ABAT deficiency phenotype includes psychomotor retardation, hypotonia, hyperreflexia, lethargy, refractory seizures, and EEG abnormalities.4-aminobutyrate aminotransferase (ABAT) is responsible for catabolism of gamma-aminobutyric acid (GABA), an important, mostly inhibitory neurotransmitter in the central nervous system, into succinic semialdehyde. The active enzyme is a homodimer of 50-kD subunits complexed to pyridoxal-5-phosphate. The protein sequence is over 95% similar to the pig protein. GABA is estimated to be present in nearly one-third of human synapses. ABAT in liver and brain is controlled by 2 codominant alleles with a frequency in a Caucasian population of 0.56 and 0.44. The ABAT deficiency phenotype includes psychomotor retardation, hypotonia, hyperreflexia, lethargy, refractory seizures, and EEG abnormalities. Multiple alternatively spliced transcript variants encoding the same protein isoform have been found for this gene.
UOM: 1 * 50 µG


Artikel-Nr: (PRSI30-379)
Lieferant: ProSci Inc.
Hersteller Artikel Nummer : 30-379
Beschreibung: Glutamic-oxaloacetic transaminase is a pyridoxal phosphate-dependent enzyme which exists in cytoplasmic and mitochondrial forms, GOT1 and GOT2, respectively. GOT plays a role in amino acid metabolism and the urea and tricarboxylic acid cycles. The two enzymes are homodimeric and show close homology.Glutamic-oxaloacetic transaminase is a pyridoxal phosphate-dependent enzyme which exists in cytoplasmic and mitochondrial forms, GOT1 and GOT2, respectively. GOT plays a role in amino acid metabolism and the urea and tricarboxylic acid cycles. The two enzymes are homodimeric and show close homology. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.
UOM: 1 * 100 µG


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Artikel-Nr: (PRSI25-873)
Lieferant: ProSci Inc.
Hersteller Artikel Nummer : 25-873
Beschreibung: Glutamic-oxaloacetic transaminase is a pyridoxal phosphate-dependent enzyme which exists in cytoplasmic and inner-membrane mitochondrial forms, GOT1 and GOT2, respectively. GOT plays a role in amino acid metabolism and the urea and tricarboxylic acid cycles. The two enzymes are homodimeric and show close homology.Glutamic-oxaloacetic transaminase is a pyridoxal phosphate-dependent enzyme which exists in cytoplasmic and inner-membrane mitochondrial forms, GOT1 and GOT2, respectively. GOT plays a role in amino acid metabolism and the urea and tricarboxylic acid cycles. The two enzymes are homodimeric and show close homology. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.
UOM: 1 * 50 µG


Artikel-Nr: (PRSI26-849)
Lieferant: ProSci Inc.
Hersteller Artikel Nummer : 26-849
Beschreibung: The enzyme system for cleavage of glycine (glycine cleavage system; EC 2.1.2.10), which is confined to the mitochondria, is composed of 4 protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase). Glycine encephalopathy (GCE) may be due to a defect in any one of these enzymes.The enzyme system for cleavage of glycine (glycine cleavage system; EC 2.1.2.10), which is confined to the mitochondria, is composed of 4 protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase; MIM 238300), H protein (a lipoic acid-containing protein; MIM 238330), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase; MIM 238331). Glycine encephalopathy (GCE; MIM 605899) may be due to a defect in any one of these enzymes.
UOM: 1 * 50 µG


Artikel-Nr: (PRSI49-368)
Lieferant: ProSci Inc.
Hersteller Artikel Nummer : 49-368
Beschreibung: Glutamic-oxaloacetic transaminase is a pyridoxal phosphate-dependent enzyme which exists in cytoplasmic and inner-membrane mitochondrial forms, GOT1 and GOT2, respectively. GOT plays a role in amino acid metabolism and the urea and tricarboxylic acid cycles. The two enzymes are homodimeric and show close homology.
UOM: 1 * 50 µl


Artikel-Nr: (PRSI29-877)
Lieferant: ProSci Inc.
Hersteller Artikel Nummer : 29-877
Beschreibung: Glutamic-oxaloacetic transaminase is a pyridoxal phosphate-dependent enzyme which exists in cytoplasmic and inner-membrane mitochondrial forms, GOT1 and GOT2, respectively. GOT plays a role in amino acid metabolism and the urea and tricarboxylic acid cycles. The two enzymes are homodimeric and show close homology.Glutamic-oxaloacetic transaminase is a pyridoxal phosphate-dependent enzyme which exists in cytoplasmic and inner-membrane mitochondrial forms, GOT1 and GOT2, respectively. GOT plays a role in amino acid metabolism and the urea and tricarboxylic acid cycles. The two enzymes are homodimeric and show close homology.
UOM: 1 * 100 µG


Artikel-Nr: (PRSI92-667)
Lieferant: ProSci Inc.
Hersteller Artikel Nummer : 92-667
Beschreibung: Tryptophan synthase is a multienzyme alpha2 beta 2 complex composed of two protein subunit. Tryptophan synthase catalyses the last two steps in the synthesis of L-tryptophan (L-Trp). The alpha-subunit catalyses cleavage of 3-indole-d-glycerol 3′-phosphate (IGP) to give indole and D-glyceraldehyde 3′-phosphate (G3P). Indole is then transferred through a 25-Å hydrophobic tunnel to the beta -subunit. The beta 2 subunit contains pyridoxal 5'-phosphate and catalyses several pyridoxal 5'-phosphate-dependent reactions, including/3-elimination reactions 6 and a thiol-dependent transamination reaction. This enzyme is commonly found in Eubacteria, Archaebacteria, Protista, Fungi, and Plantae, but is absent from Animalia. As humans do not have tryptophan synthase, this enzyme has been explored as a potential drug target.
UOM: 1 * 50 µG


Artikel-Nr: (PRSI92-622)
Lieferant: ProSci Inc.
Hersteller Artikel Nummer : 92-622
Beschreibung: Tryptophan synthase is an enzyme that catalyses the final two steps in the biosynthesis of tryptophan. It is commonly found in Eubacteria, Archaebacteria, Protista, Fungi, and Plantae, but is absent from animals such as humans. Tryptophan synthase typically exists as an alpha- beta beta - alpha complex.The alpha subunit is responsible for the aldol cleavage of indoleglycerol phosphate to indole and glyceraldehyde 3-phosphate: L-serine + 1-C-(indol-3-yl)glycerol 3-phosphate = L-tryptophan + D-glyceraldehyde 3-phosphate + H₂O.The beta subunits catalyse the irreversible condensation of indole and serine to form tryptophan in a pyridoxal phosphate (PLP) dependent reaction. Their assembly into a complex leads to structural changes in both subunits resulting in reciprocal activation.
UOM: 1 * 50 µG


Artikel-Nr: (PRSI92-623)
Lieferant: ProSci Inc.
Hersteller Artikel Nummer : 92-623
Beschreibung: Tryptophan synthase is an enzyme that catalyses the final two steps in the biosynthesis of tryptophan. It is commonly found in Eubacteria, Archaebacteria, Protista, Fungi, and Plantae, but is absent from animals such as humans. Tryptophan synthase typically exists as an alpha- beta beta - alpha complex.The alpha subunit is responsible for the aldol cleavage of indoleglycerol phosphate to indole and glyceraldehyde 3-phosphate: L-serine + 1-C-(indol-3-yl)glycerol 3-phosphate = L-tryptophan + D-glyceraldehyde 3-phosphate + H₂O.The beta subunits catalyse the irreversible condensation of indole and serine to form tryptophan in a pyridoxal phosphate (PLP) dependent reaction. Their assembly into a complex leads to structural changes in both subunits resulting in reciprocal activation.
UOM: 1 * 50 µG


Artikel-Nr: (PRSI26-155)
Lieferant: ProSci Inc.
Hersteller Artikel Nummer : 26-155
Beschreibung: Serine palmitoyltransferase, which consists of two different subunits, is the key enzyme in sphingolipid biosynthesis. It converts L-serine and palmitoyl-CoA to 3-oxosphinganine with pyridoxal 5'-phosphate as a cofactor. SPTLC1 is the long chain base subunit 1 of serine palmitoyltransferase. Mutations in SPTLC1 gene were identified in patients with hereditary sensory neuropathy type 1. Serine palmitoyltransferase, which consists of two different subunits, is the key enzyme in sphingolipid biosynthesis. It converts L-serine and palmitoyl-CoA to 3-oxosphinganine with pyridoxal 5'-phosphate as a cofactor. The product of this gene is the long chain base subunit 1 of serine palmitoyltransferase. Mutations in this gene were identified in patients with hereditary sensory neuropathy type 1. Alternatively spliced variants encoding different isoforms have been identified.
UOM: 1 * 50 µG


Artikel-Nr: (PRSI27-792)
Lieferant: ProSci Inc.
Hersteller Artikel Nummer : 27-792
Beschreibung: Iron-sulfur clusters are required for the function of many cellular enzymes. The protein encoded by this gene supplies inorganic sulfur to these clusters by removing the sulfur from cysteine, creating alanine in the process. This gene uses alternate in-frame translation initiation sites to generate mitochondrial forms and cytoplasmic/nuclear forms. Selection of the alternative initiation sites is determined by the cytosolic pH. The encoded protein belongs to the class-V family of pyridoxal phosphate-dependent aminotransferases. Two transcript variants encoding two different isoforms have been found for this gene.
UOM: 1 * 100 µG


Artikel-Nr: (PRSI6303)
Lieferant: ProSci Inc.
Hersteller Artikel Nummer : 6303
Beschreibung: SPT1 Antibody: Serine palmitoyltransferase, which consists of two different subunits, is the key enzyme in sphingolipid biosynthesis. It converts L-serine and palmitoyl-CoA to 3-oxosphinganine with pyridoxal 5'-phosphate as a cofactor. SPT1 is the long chain base subunit 1 of mammalian serine palmitoyltransferase. SPT1 is not catalytically active but is necessary for the stabilization of the SPT2 subunit and anchoring the holoenzyme to the cytosolic face of the endoplasmic reticulum. Missense mutations in this gene have been identified in patients with hereditary sensory neuropathy type 1 (HSAN1). These mutations induce a shift in the substrate specificity of the holoenzyme, leading to the formation and accumulation of two neurotoxic sphingolipids.
UOM: 1 * 100 µG


Artikel-Nr: (PRSI49-260)
Lieferant: ProSci Inc.
Hersteller Artikel Nummer : 49-260
Beschreibung: ALAS-1 (EC 2.3.1.37) is a nuclear-encoded mitochondrial matrix enzymecatalyzing the condensation of glycine with succinyl-CoA to form delta-amino levulinate, CO2 and CoA. It regulates the first and rate-limiting step of heme biosynthetic pathway. It is one of the two isoforms of ALAS and is a pyridoxal-5 phosphate dependant housekeeping enzyme. It is ubiquitously expressed and is responsible of providing heme for cytochromes and other hemoproteins. ALAS1 in liver undergoes negative feedback regulation by heme. Transcription of ALAS1 gene is stimulated by cAMP and respiratory uncoupling whereas Phorbol esters and insulin repress ALAS1 gene expression. ALAS1 is suggested to be involved in reciprocal regulation of Heme biosynthesis and circadian clock suggesting a potential target for treatment of circadian disorders. ALAS1 gene expression is down regulated in Acute Liver failure resulting in altered heme metabolism and liver function.
UOM: 1 * 50 µG


Artikel-Nr: (PRSI6305)
Lieferant: ProSci Inc.
Hersteller Artikel Nummer : 6305
Beschreibung: SPT2 Antibody: Serine palmitoyltransferase (SPT), which consists of two different subunits, is the key enzyme in sphingolipid biosynthesis. It converts L-serine and palmitoyl-CoA to 3-oxosphinganine with pyridoxal 5'-phosphate as a cofactor. SPT2 is the long chain base subunit 2 of mammalian serine palmitoyltransferase. SPT2 is catalytically active but needs its related protein SPT1 for its stabilization and anchoring the holoenzyme to the cytosolic face of the endoplasmic reticulum. As in the case with SPT1, mutations in the SPT2 gene can cause hereditary sensory and autonomic neuropathy type I (HSAN-I), resulting from a loss of SPT activity as well as the accumulation of the atypical and neurotoxic sphingoid metabolite 1-deoxy-sphinganine.
UOM: 1 * 100 µG


Artikel-Nr: (PRSI56-722)
Lieferant: ProSci Inc.
Hersteller Artikel Nummer : 56-722
Beschreibung: The degradation of L-threonine to glycine consists of a two-step biochemical pathway involving the enzymes L-threonine dehydrogenase and 2-amino-3-ketobutyrate coenzyme A ligase. L-Threonine is first converted into 2-amino-3-ketobutyrate by L-threonine dehydrogenase. This gene encodes the second enzyme in this pathway, which then catalyzes the reaction between 2-amino-3-ketobutyrate and coenzyme A to form glycine and acetyl-CoA. The encoded enzyme is considered a class II pyridoxal-phosphate-dependent aminotransferase. Alternate splicing results in multiple transcript variants. A pseudogene of this gene is found on chromosome 14.
UOM: 1 * 400 µl

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Artikel-Nr: (8.00026.9020)
Lieferant: Merck
Hersteller Artikel Nummer : 8.00026.9020
Beschreibung: Acetamidin Hydrochlorid
UOM: 1 * 20 kg


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