Argininosuccinate Lyase (ASL) antibody (Cy5)

Details for Product No. ABIN1704717
Antigen
Reactivity
Human, Mouse (Murine), Rat (Rattus)
72
37
28
6
6
6
6
6
5
4
3
3
3
3
3
3
2
Host
Rabbit
64
8
Clonality
Polyclonal
Conjugate
Cy5
4
3
2
2
2
2
2
2
2
1
1
1
1
1
1
1
Application
Immunofluorescence (Paraffin-embedded Sections) (IF (p))
41
13
12
12
11
9
7
5
3
2
2
2
1
Options
Immunogen KLH conjugated synthetic peptide derived from human ASL/Argininosuccinate Lyase
Isotype IgG
Purification Purified by Protein A.
Antigen
Alternative Name Asl/Argininosuccinate Lyase (ASL Antibody Abstract)
Background

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).

Synonyms: Argininosuccinase, Argininosuccinate lyase, Arginosuccinase, ARLY_HUMAN, ASAL, ASL, EC 4.3.2.1.

Gene ID 435
Pathways Response to Growth Hormone Stimulus
Application Notes IF(IHC-P) 1:50-200
Restrictions For Research Use only
Format Liquid
Concentration 1 μg/μL
Buffer Aqueous buffered solution containing 1 % BSA, 50 % glycerol and 0.09 % sodium azide.
Preservative Sodium azide
Precaution of Use This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
Storage 4 °C
Storage Comment Store at 4°C
Expiry Date 12 months