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Descripción: HGD is a 445 amino acid protein that belongs to the homogentisate dioxygenase family and is involved in the pathway of amino acid degradation. Expressed at high levels in kidney, colon, liver, prostate and small intestine, HGD uses iron as a cofactor to catalyse the oxygen-dependent conversion of homogentisate to 4-maleylacetoacetate, a reaction that is the fourth step in the creation of L-phenylalanine from fumarate and acetoacetic acid. Defects in the gene encoding HGD are the cause of alkaptonuria (AKU), an autosomal recessive disorder that is characterised by urine that turns dark on standing and alkalinisation, black ochronotic pigmentation of cartilage and collagenous tissues and spine arthritis.

Numero del catalogo: BOSSBS-15472R-A350

UOM: 1 * 100 µl

Proveedor: Bioss

Promoción

Descripción: 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.

Numero del catalogo: BOSSBS-0136R-FITC

UOM: 1 * 100 µl

Proveedor: Bioss

Promoción

Descripción: HGD is a 445 amino acid protein that belongs to the homogentisate dioxygenase family and is involved in the pathway of amino acid degradation. Expressed at high levels in kidney, colon, liver, prostate and small intestine, HGD uses iron as a cofactor to catalyse the oxygen-dependent conversion of homogentisate to 4-maleylacetoacetate, a reaction that is the fourth step in the creation of L-phenylalanine from fumarate and acetoacetic acid. Defects in the gene encoding HGD are the cause of alkaptonuria (AKU), an autosomal recessive disorder that is characterised by urine that turns dark on standing and alkalinisation, black ochronotic pigmentation of cartilage and collagenous tissues and spine arthritis.

Numero del catalogo: BOSSBS-15472R-CY3

UOM: 1 * 100 µl

Proveedor: Bioss

Promoción

Descripción: HGD is a 445 amino acid protein that belongs to the homogentisate dioxygenase family and is involved in the pathway of amino acid degradation. Expressed at high levels in kidney, colon, liver, prostate and small intestine, HGD uses iron as a cofactor to catalyse the oxygen-dependent conversion of homogentisate to 4-maleylacetoacetate, a reaction that is the fourth step in the creation of L-phenylalanine from fumarate and acetoacetic acid. Defects in the gene encoding HGD are the cause of alkaptonuria (AKU), an autosomal recessive disorder that is characterised by urine that turns dark on standing and alkalinisation, black ochronotic pigmentation of cartilage and collagenous tissues and spine arthritis.

Numero del catalogo: BOSSBS-15472R-CY7

UOM: 1 * 100 µl

Proveedor: Bioss

Promoción

Descripción: GTP cyclohydrolase I (also designated dopa-responsive dystonia) catalyses 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 synthesise tetrahydrobiopterin in vivo from GTP through several enzymatic reactions.

Numero del catalogo: BOSSBS-0136R-A680

UOM: 1 * 100 µl

Proveedor: Bioss

Promoción

Descripción: DNAJB12 belongs to the evolutionarily conserved DNAJ/HSP40 family of proteins, which regulate molecular chaperone activity by stimulating ATPase activity. DNAJ proteins may have up to 3 distinct domains: a conserved 70-amino acid J domain, usually at the N terminus; a glycine/phenylalanine (G/F)-rich region; and a cysteine-rich domain containing 4 motifs resembling a zinc finger domain (Ohtsuka and Hata, 2000 [PubMed 11147971]).[supplied by OMIM, Mar 2008]

Numero del catalogo: BOSSBS-14371R-A750

UOM: 1 * 100 µl

Proveedor: Bioss

Promoción

Descripción: DNAJB12 belongs to the evolutionarily conserved DNAJ/HSP40 family of proteins, which regulate molecular chaperone activity by stimulating ATPase activity. DNAJ proteins may have up to 3 distinct domains: a conserved 70-amino acid J domain, usually at the N terminus; a glycine/phenylalanine (G/F)-rich region; and a cysteine-rich domain containing 4 motifs resembling a zinc finger domain (Ohtsuka and Hata, 2000 [PubMed 11147971]).[supplied by OMIM, Mar 2008]

Numero del catalogo: BOSSBS-14371R-A680

UOM: 1 * 100 µl

Proveedor: Bioss

Promoción

Descripción: HGD is a 445 amino acid protein that belongs to the homogentisate dioxygenase family and is involved in the pathway of amino acid degradation. Expressed at high levels in kidney, colon, liver, prostate and small intestine, HGD uses iron as a cofactor to catalyse the oxygen-dependent conversion of homogentisate to 4-maleylacetoacetate, a reaction that is the fourth step in the creation of L-phenylalanine from fumarate and acetoacetic acid. Defects in the gene encoding HGD are the cause of alkaptonuria (AKU), an autosomal recessive disorder that is characterised by urine that turns dark on standing and alkalinisation, black ochronotic pigmentation of cartilage and collagenous tissues and spine arthritis.

Numero del catalogo: BOSSBS-15472R-A680

UOM: 1 * 100 µl

Proveedor: Bioss

Promoción

Descripción: HGD is a 445 amino acid protein that belongs to the homogentisate dioxygenase family and is involved in the pathway of amino acid degradation. Expressed at high levels in kidney, colon, liver, prostate and small intestine, HGD uses iron as a cofactor to catalyse the oxygen-dependent conversion of homogentisate to 4-maleylacetoacetate, a reaction that is the fourth step in the creation of L-phenylalanine from fumarate and acetoacetic acid. Defects in the gene encoding HGD are the cause of alkaptonuria (AKU), an autosomal recessive disorder that is characterised by urine that turns dark on standing and alkalinisation, black ochronotic pigmentation of cartilage and collagenous tissues and spine arthritis.

Numero del catalogo: BOSSBS-15472R-A555

UOM: 1 * 100 µl

Proveedor: Bioss

Promoción

Descripción: Aminoacyl-tRNA synthetases consist of a family of enzymes that catalyze the specific aminoacylation of tRNA by their cognate amino acid in the initial step of ribosome-dependent protein biosynthesis. FARSLA, also known as FRSA, CML33, FARSL or PheHA (phenylalanyl-tRNA synthetase, alpha subunit), is a member of the class-II aminoacyl-tRNA synthetase family and is highly expressed in proliferating cells of bone marrow. FARSLA is a cytoplasmic phenylalanine-tRNA synthetase that functions as a heterodimer consisting of a catalytic alpha-subunit and a regulatory beta-subunit. The alpha-subunit is responsible for forming the amino acid binding pocket, mediating the ATP/aminoacyl adenylate binding, and interacts with the acceptor stem of the tRNA. FARSLA functions in a cell cycle-dependent and differentiation-dependent manner.

Numero del catalogo: BOSSBS-13145R-CY7

UOM: 1 * 100 µl

Proveedor: Bioss

Promoción

Descripción: Aminoacyl-tRNA synthetases consist of a family of enzymes that catalyze the specific aminoacylation of tRNA by their cognate amino acid in the initial step of ribosome-dependent protein biosynthesis. FARSLA, also known as FRSA, CML33, FARSL or PheHA (phenylalanyl-tRNA synthetase, alpha subunit), is a member of the class-II aminoacyl-tRNA synthetase family and is highly expressed in proliferating cells of bone marrow. FARSLA is a cytoplasmic phenylalanine-tRNA synthetase that functions as a heterodimer consisting of a catalytic alpha-subunit and a regulatory beta-subunit. The alpha-subunit is responsible for forming the amino acid binding pocket, mediating the ATP/aminoacyl adenylate binding, and interacts with the acceptor stem of the tRNA. FARSLA functions in a cell cycle-dependent and differentiation-dependent manner.

Numero del catalogo: BOSSBS-13145R-A680

UOM: 1 * 100 µl

Proveedor: Bioss

Promoción

Descripción: Aminoacyl-tRNA synthetases consist of a family of enzymes that catalyze the specific aminoacylation of tRNA by their cognate amino acid in the initial step of ribosome-dependent protein biosynthesis. FARSLA, also known as FRSA, CML33, FARSL or PheHA (phenylalanyl-tRNA synthetase, alpha subunit), is a member of the class-II aminoacyl-tRNA synthetase family and is highly expressed in proliferating cells of bone marrow. FARSLA is a cytoplasmic phenylalanine-tRNA synthetase that functions as a heterodimer consisting of a catalytic alpha-subunit and a regulatory beta-subunit. The alpha-subunit is responsible for forming the amino acid binding pocket, mediating the ATP/aminoacyl adenylate binding, and interacts with the acceptor stem of the tRNA. FARSLA functions in a cell cycle-dependent and differentiation-dependent manner.

Numero del catalogo: BOSSBS-13145R-FITC

UOM: 1 * 100 µl

Proveedor: Bioss

Promoción

Descripción: Aminoacyl-tRNA synthetases consist of a family of enzymes that catalyze the specific aminoacylation of tRNA by their cognate amino acid in the initial step of ribosome-dependent protein biosynthesis. FARSLA, also known as FRSA, CML33, FARSL or PheHA (phenylalanyl-tRNA synthetase, alpha subunit), is a member of the class-II aminoacyl-tRNA synthetase family and is highly expressed in proliferating cells of bone marrow. FARSLA is a cytoplasmic phenylalanine-tRNA synthetase that functions as a heterodimer consisting of a catalytic alpha-subunit and a regulatory beta-subunit. The alpha-subunit is responsible for forming the amino acid binding pocket, mediating the ATP/aminoacyl adenylate binding, and interacts with the acceptor stem of the tRNA. FARSLA functions in a cell cycle-dependent and differentiation-dependent manner.

Numero del catalogo: BOSSBS-13145R-A647

UOM: 1 * 100 µl

Proveedor: Bioss

Promoción

Descripción: Aminoacyl-tRNA synthetases consist of a family of enzymes that catalyze the specific aminoacylation of tRNA by their cognate amino acid in the initial step of ribosome-dependent protein biosynthesis. FARSLA, also known as FRSA, CML33, FARSL or PheHA (phenylalanyl-tRNA synthetase, alpha subunit), is a member of the class-II aminoacyl-tRNA synthetase family and is highly expressed in proliferating cells of bone marrow. FARSLA is a cytoplasmic phenylalanine-tRNA synthetase that functions as a heterodimer consisting of a catalytic alpha-subunit and a regulatory beta-subunit. The alpha-subunit is responsible for forming the amino acid binding pocket, mediating the ATP/aminoacyl adenylate binding, and interacts with the acceptor stem of the tRNA. FARSLA functions in a cell cycle-dependent and differentiation-dependent manner.

Numero del catalogo: BOSSBS-13145R-CY3

UOM: 1 * 100 µl

Proveedor: Bioss

Promoción

Descripción: Aminoacyl-tRNA synthetases consist of a family of enzymes that catalyze the specific aminoacylation of tRNA by their cognate amino acid in the initial step of ribosome-dependent protein biosynthesis. FARSLA, also known as FRSA, CML33, FARSL or PheHA (phenylalanyl-tRNA synthetase, alpha subunit), is a member of the class-II aminoacyl-tRNA synthetase family and is highly expressed in proliferating cells of bone marrow. FARSLA is a cytoplasmic phenylalanine-tRNA synthetase that functions as a heterodimer consisting of a catalytic alpha-subunit and a regulatory beta-subunit. The alpha-subunit is responsible for forming the amino acid binding pocket, mediating the ATP/aminoacyl adenylate binding, and interacts with the acceptor stem of the tRNA. FARSLA functions in a cell cycle-dependent and differentiation-dependent manner.

Numero del catalogo: BOSSBS-13145R-A555

UOM: 1 * 100 µl

Proveedor: Bioss

Promoción

Descripción: Aminoacyl-tRNA synthetases consist of a family of enzymes that catalyze the specific aminoacylation of tRNA by their cognate amino acid in the initial step of ribosome-dependent protein biosynthesis. FARSLA, also known as FRSA, CML33, FARSL or PheHA (phenylalanyl-tRNA synthetase, alpha subunit), is a member of the class-II aminoacyl-tRNA synthetase family and is highly expressed in proliferating cells of bone marrow. FARSLA is a cytoplasmic phenylalanine-tRNA synthetase that functions as a heterodimer consisting of a catalytic alpha-subunit and a regulatory beta-subunit. The alpha-subunit is responsible for forming the amino acid binding pocket, mediating the ATP/aminoacyl adenylate binding, and interacts with the acceptor stem of the tRNA. FARSLA functions in a cell cycle-dependent and differentiation-dependent manner.

Numero del catalogo: BOSSBS-13145R-A488

UOM: 1 * 100 µl

Proveedor: Bioss

Promoción