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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
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-BOSSBS-12515R-A750

Anti-ASL/Argininosuccinate Lyase Rabbit Polyclonal Antibody (Alexa Fluor® 750)

Catalog Number: (BOSSBS-12515R-A750)
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-12515R-FITC

Anti-ASL Rabbit Polyclonal Antibody (FITC (Fluorescein Isothiocyanate))

Catalog Number: (BOSSBS-12515R-FITC)
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-SPCMF1096-12KG

Fumaric acid NF

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

Image Unavailable-EHERC13955500

Fumaric acid

Catalog Number: (EHERC13955500)
Supplier: EHRENSTORFER
Description: Fumaric acid
UOM: 1 * 0,25 g

Certificates


Image Unavailable-BOSSBS-12515R-HRP

Anti-ASL Rabbit Polyclonal Antibody (HRP (Horseradish Peroxidase))

Catalog Number: (BOSSBS-12515R-HRP)
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-A350

Anti-ASL Rabbit Polyclonal Antibody (Alexa Fluor® 350)

Catalog Number: (BOSSBS-12515R-A350)
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-SIAL03761-500G

Fumaric acid, Sigma-Aldrich®

Catalog Number: (SIAL03761-500G)
Supplier: Merck
Description: Fumaric acid, Sigma-Aldrich®
UOM: 1 * 500 g
Image Unavailable-SIALW248800-1KG-K

Fumaric acid, Sigma-Aldrich®

Supplier: Merck
Description: Fumaric acid, Sigma-Aldrich®

Image Unavailable-MOLE15604498-1KG

Fumaric acid

Catalog Number: (MOLE15604498-1KG)
Supplier: Molekula
Description: Fumaric acid
UOM: 1 * 1 kg
Image Unavailable-SIAL47910-100G

Fumaric acid, Sigma-Aldrich®

Supplier: Merck
Description: Fumaric acid, Sigma-Aldrich®

Image Unavailable-SIALF1506-100G

Disodium fumarate, Sigma-Aldrich®

Supplier: Merck
Description: Disodium fumarate, Sigma-Aldrich®

Product Image-ACRO117890025

Diethylene glycol monoethyl ether 98+%

Supplier: Thermo Fisher Scientific
Description: Diethylene glycol monoethyl ether 98+%

MSDS Certificates

Product Image-103426R

2-Ethoxyethanol ≥99.5%, AnalaR NORMAPUR® analytical reagent

Catalog Number: (103426R)
Supplier: VWR Chemicals
Description: 2-Ethoxyethanol ≥99.5%, AnalaR NORMAPUR® analytical reagent
UOM: 1 * 2,5 L

MSDS Certificates


Image Unavailable-B24755.36

2-Ethoxyphenol ≥98%

Catalog Number: (B24755.36)
Supplier: Thermo Fisher Scientific
Description: 2-Ethoxyphenol ≥98%
UOM: 1 * 500 g

MSDS Certificates


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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 at +43 1 97002 - 0.
Dual use goods can only be delivered within the European Union.
Dual use goods can only be delivered within the European Union.
This product has been blocked by your organization. Please contact your purchasing department for more information.
The original product is no longer available. The replacement shown is available.
This product is no longer available. Alternatives may be available by searching with the VWR Catalog Number listed above. If you need further assistance, please call VWR Customer Service at +43 1 97002 - 0.
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