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Image Unavailable-BOSSBS-15472R-CY7

Anti-HGD Rabbit Polyclonal Antibody (Cy7)

Catalog Number: (BOSSBS-15472R-CY7)
Supplier: Bioss
Description: 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.
UOM: 1 * 100 µl
Image Unavailable-BOSSBS-15472R-CY3

Anti-HGD Rabbit Polyclonal Antibody (Cy3)

Catalog Number: (BOSSBS-15472R-CY3)
Supplier: Bioss
Description: 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.
UOM: 1 * 100 µl

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Image Unavailable-SIAL16006-250G

SP Brij® S2 MBAL, Sigma-Aldrich®

Catalog Number: (SIAL16006-250G)
Supplier: Merck
Description: Main component: diethylene glycol octadecyl ether
UOM: 1 * 250 g
Image Unavailable-EHERC12606600

Diethyl fumarate

Catalog Number: (EHERC12606600)
Supplier: EHRENSTORFER
Description: Diethyl fumarate
UOM: 1 * 0,25 g
Image Unavailable-SIALA3134-10G

3-Aminopropionitrile fumarate, Sigma-Aldrich®

Supplier: Merck
Description: 3-Aminopropionitrile fumarate, Sigma-Aldrich®

Image Unavailable-BOSSBS-12515R-CY7

Anti-ASL Rabbit Polyclonal Antibody (Cy7®)

Catalog Number: (BOSSBS-12515R-CY7)
Supplier: Bioss
Description: ASL is a member of the lyase 1 family of proteins and is predominantly expressed in the liver. Localizing to the cytoplasm and existing as a homotetramer, ASL catalyzes the hydrolytic cleavage of argininosuccinic acid (ASA) to fumarate and arginine, an essential step of the urea cycle which is crucial for the detoxification of ammonia. This reaction is also involved in the biosynthesis of arginine. In addition, ASL shares high sequence homology with the avian and reptilian eye lens protein, d-crystallin. Mutations in the gene encoding ASL leads to an accumulation of ASA in body fluids and results in Arginosuc-cinic aciduria (ASAuria), an autosomal recessive disorder that is characterized by hyperammonemia, liver enlargement, convulsions, physical and mental retardation, episodic unconsciousness and dry and brittle hair showing trich-orrhexis nodosa (weak points or nodes in the hair shaft).
UOM: 1 * 100 µl
Image Unavailable-BOSSBS-12515R-CY3

Anti-ASL Rabbit Polyclonal Antibody (Cy3®)

Catalog Number: (BOSSBS-12515R-CY3)
Supplier: Bioss
Description: ASL is a member of the lyase 1 family of proteins and is predominantly expressed in the liver. Localizing to the cytoplasm and existing as a homotetramer, ASL catalyzes the hydrolytic cleavage of argininosuccinic acid (ASA) to fumarate and arginine, an essential step of the urea cycle which is crucial for the detoxification of ammonia. This reaction is also involved in the biosynthesis of arginine. In addition, ASL shares high sequence homology with the avian and reptilian eye lens protein, d-crystallin. Mutations in the gene encoding ASL leads to an accumulation of ASA in body fluids and results in Arginosuc-cinic aciduria (ASAuria), an autosomal recessive disorder that is characterized by hyperammonemia, liver enlargement, convulsions, physical and mental retardation, episodic unconsciousness and dry and brittle hair showing trich-orrhexis nodosa (weak points or nodes in the hair shaft).
UOM: 1 * 100 µl
Image Unavailable-BOSSBS-12515R-A680

Anti-ASL/Argininosuccinate Lyase Rabbit Polyclonal Antibody (Alexa Fluor® 680)

Catalog Number: (BOSSBS-12515R-A680)
Supplier: Bioss
Description: ASL is a member of the lyase 1 family of proteins and is predominantly expressed in the liver. Localizing to the cytoplasm and existing as a homotetramer, ASL catalyses the hydrolytic cleavage of argininosuccinic acid (ASA) to fumarate and arginine, an essential step of the urea cycle which is crucial for the detoxification of ammonia. This reaction is also involved in the biosynthesis of arginine. In addition, ASL shares high sequence homology with the avian and reptilian eye lens protein, d-crystallin. Mutations in the gene encoding ASL leads to an accumulation of ASA in body fluids and results in Arginosuc-cinic aciduria (ASAuria), an autosomal recessive disorder that is characterised by hyperammonemia, liver enlargement, convulsions, physical and mental retardation, episodic unconsciousness and dry and brittle hair showing trich-orrhexis nodosa (weak points or nodes in the hair shaft).
UOM: 1 * 100 µl
Image Unavailable-BOSSBS-15472R-CY5

Anti-HGD Rabbit Polyclonal Antibody (Cy5)

Catalog Number: (BOSSBS-15472R-CY5)
Supplier: Bioss
Description: 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.
UOM: 1 * 100 µl
Image Unavailable-MOLE42559860-25G

N-Octadecyl-N,N-dimethyl-3-ammonio-1-propanesulphonate(sulphobetaine-18, SB-18)

Catalog Number: (MOLE42559860-25G)
Supplier: Molekula
Description: N-Octadecyl-N,N-dimethyl-3-ammonio-1-propanesulphonate(sulphobetaine-18, SB-18)
UOM: 1 * 25 g

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Image Unavailable-BOSSBS-15472R-A350

Anti-HGD Rabbit Polyclonal Antibody (ALEXA FLUOR® 350)

Catalog Number: (BOSSBS-15472R-A350)
Supplier: Bioss
Description: 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.
UOM: 1 * 100 µl
Image Unavailable-BOSSBS-15472R-A750

Anti-HGD Rabbit Polyclonal Antibody (ALEXA FLUOR® 750)

Catalog Number: (BOSSBS-15472R-A750)
Supplier: Bioss
Description: 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.
UOM: 1 * 100 µl
Image Unavailable-BOSSBS-12515R-A647

Anti-ASL Rabbit Polyclonal Antibody (Alexa Fluor® 647)

Catalog Number: (BOSSBS-12515R-A647)
Supplier: Bioss
Description: ASL is a member of the lyase 1 family of proteins and is predominantly expressed in the liver. Localizing to the cytoplasm and existing as a homotetramer, ASL catalyzes the hydrolytic cleavage of argininosuccinic acid (ASA) to fumarate and arginine, an essential step of the urea cycle which is crucial for the detoxification of ammonia. This reaction is also involved in the biosynthesis of arginine. In addition, ASL shares high sequence homology with the avian and reptilian eye lens protein, d-crystallin. Mutations in the gene encoding ASL leads to an accumulation of ASA in body fluids and results in Arginosuc-cinic aciduria (ASAuria), an autosomal recessive disorder that is characterized by hyperammonemia, liver enlargement, convulsions, physical and mental retardation, episodic unconsciousness and dry and brittle hair showing trich-orrhexis nodosa (weak points or nodes in the hair shaft).
UOM: 1 * 100 µl
Image Unavailable-SPCMF1096-2.5KG

Fumaric acid NF

Supplier: Spectrum Chemical
Description: BSE/TSE statement (available upon request)
Allergen statement (available upon request)

Image Unavailable-CAYM14714-100

Dimethyl fumarate

Supplier: Cayman Chemical
Description: Dimethyl fumarate

Image Unavailable-BOSSBS-12515R-A488

Anti-ASL Rabbit Polyclonal Antibody (Alexa Fluor® 488)

Catalog Number: (BOSSBS-12515R-A488)
Supplier: Bioss
Description: ASL is a member of the lyase 1 family of proteins and is predominantly expressed in the liver. Localizing to the cytoplasm and existing as a homotetramer, ASL catalyzes the hydrolytic cleavage of argininosuccinic acid (ASA) to fumarate and arginine, an essential step of the urea cycle which is crucial for the detoxification of ammonia. This reaction is also involved in the biosynthesis of arginine. In addition, ASL shares high sequence homology with the avian and reptilian eye lens protein, d-crystallin. Mutations in the gene encoding ASL leads to an accumulation of ASA in body fluids and results in Arginosuc-cinic aciduria (ASAuria), an autosomal recessive disorder that is characterized by hyperammonemia, liver enlargement, convulsions, physical and mental retardation, episodic unconsciousness and dry and brittle hair showing trich-orrhexis nodosa (weak points or nodes in the hair shaft).
UOM: 1 * 100 µl
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Stock for this item is limited, but may be available in a warehouse close to you. Please make sure that you are logged in to the site so that available stock can be displayed. If the call is still displayed and you need assistance, please call us on 0800 22 33 44
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Additional Documentation may be needed to purchase this item. A VWR representative will contact you if needed.
This product has been blocked by your organisation. Please contact your purchasing department for more information.
The original product is no longer available. The replacement shown is available.
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