Anti-ASL 항체 (CAB6357)

Antibody Name:	Anti-ASL Antibody
Antibody SKU:	CAB6357
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human ASL (NP_000039.2).

Application:	WB IHC IF
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:100
Reactivity:	Human, Mouse, Rat
Positive Samples:	Mouse liver, Mouse brain, Mouse kidney, Rat liver

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human ASL (NP_000039.2).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	MASE SGKL WGGR FVGA VDPI MEKF NASI AYDR HLWE VDVQ GSKA YSRG LEKA GLLT KAEM DQIL HGLD KVAE EWAQ GTFK LNSN DEDI HTAN ERRL KELI GATA GKLH TGRS RNDQ VVTD LRLW MRQT CSTL SGLL WELI RTMV DRAE AERD VLFP GYTH LQRA QPIR WSHW ILSH AVAL TRDS ERLL EVRK RINV LPLG SGAI AGNP LGVD RELL RAEL NFGA ITLN SMDA TSER DFVA EFLF WASL CMTH LSRM AEDL ILYC TKEF SFVQ LSDA YSTG SSLM PQKK NPDS LELI RSKA
Gene ID:	435
Uniprot:	P04424
Cellular Location:
Calculated MW:	48kDa/49kDa/51kDa
Observed MW:	49KDa

Synonyms:

ASL, ASAL

Background:

This gene encodes a member of the lyase 1 family. The encoded protein forms a cytosolic homotetramer and primarily catalyzes the reversible hydrolytic cleavage of argininosuccinate into arginine and fumarate, an essential step in the liver in detoxifying ammonia via the urea cycle. Mutations in this gene result in the autosomal recessive disorder argininosuccinic aciduria, or argininosuccinic acid lyase deficiency. A nontranscribed pseudogene is also located on the long arm of chromosome 22. Alternatively spliced transcript variants encoding different isoforms have been described.

UniProt Protein Function:	ASL: Defects in ASL are the cause of arginosuccinic aciduria (ARGINSA). An autosomal recessive disorder of the urea cycle. The disease is characterized by mental and physical retardation, liver enlargement, skin lesions, dry and brittle hair showing trichorrhexis nodosa microscopically and fluorescing red, convulsions, and episodic unconsciousness. Belongs to the lyase 1 family. Argininosuccinate lyase subfamily.
UniProt Protein Details:	Protein type:Lyase; Amino Acid Metabolism - alanine, aspartate and glutamate; EC 4.3.2.1; Amino Acid Metabolism - arginine and proline Chromosomal Location of Human Ortholog: 7q11.21 Cellular Component: cytoplasm; cytosol Molecular Function:argininosuccinate lyase activity Biological Process: arginine catabolic process; arginine biosynthetic process via ornithine; internal protein amino acid acetylation; urea cycle Disease: Argininosuccinic Aciduria
NCBI Summary:	This gene encodes a member of the lyase 1 family. The encoded protein forms a cytosolic homotetramer and primarily catalyzes the reversible hydrolytic cleavage of argininosuccinate into arginine and fumarate, an essential step in the liver in detoxifying ammonia via the urea cycle. Mutations in this gene result in the autosomal recessive disorder argininosuccinic aciduria, or argininosuccinic acid lyase deficiency. A nontranscribed pseudogene is also located on the long arm of chromosome 22. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2008]
UniProt Code:	P04424
NCBI GenInfo Identifier:	124028641
NCBI Gene ID:	435
NCBI Accession:	P04424.4
UniProt Secondary Accession:	P04424,Q6LDS5, Q96HS2, E7EMI0, E9PE48,
UniProt Related Accession:	P04424
Molecular Weight:	464
NCBI Full Name:	Argininosuccinate lyase
NCBI Synonym Full Names:	argininosuccinate lyase
NCBI Official Symbol:	ASL
NCBI Official Synonym Symbols:	ASAL
NCBI Protein Information:	argininosuccinate lyase; arginosuccinase; argininosuccinase
UniProt Protein Name:	Argininosuccinate lyase
UniProt Synonym Protein Names:	Arginosuccinase
UniProt Gene Name:	ASL
UniProt Entry Name:	ARLY_HUMAN

	Western blot analysis of extracts of various cell lines, using ASL antibody (CAB6357) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 1s.
	Immunohistochemistry of paraffin-embedded human liver cancer using ASL antibody (CAB6357) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded mouse kidney using ASL antibody (CAB6357) at dilution of 1:100 (40x lens).
	Immunofluorescence analysis of NIH-3T3 cells using ASL Rabbit pAb (CAB6357) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.

ELISA Kits

HumanÃ‚ ASL (ArgininosuccinateÃ‚ lyase)Ã‚ ELISAÃ‚ Kit (HUFI04621)

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)