Anti-ADSL 항체 (CAB6278)

Antibody Name:	Anti-ADSL Antibody
Antibody SKU:	CAB6278
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IF IP
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-310 of human ADSL (NP_000017.1).

Application:	WB IF IP
Recommended Dilution:	WB 1:500 - 1:2000 IF 1:20 - 1:100 IP 1:50 - 1:100
Reactivity:	Human, Mouse, Rat
Positive Samples:	HepG2, MCF7

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-310 of human ADSL (NP_000017.1).
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:	MAAG GDHG SPDS YRSP LASR YASP EMCF VFSD RYKF RTWR QLWL WLAE AEQT LGLP ITDE QIQE MKSN LENI DFKM AAEE EKRL RHDV MAHV HTFG HCCP KAAG IIHL GATS CYVG DNTD LIIL RNAL DLLL PKLA RVIS RLAD FAKE RASL PTLG FTHF QPAQ LTTV GKRC CLWI QDLC MDLQ NLKR VRDD LRFR GVKG TTGT QASF LQLF EGDD HKVE QLDK MVTE KAGF KRAF IITG QTYT RKVD IEVL SVLA SLGA SVHK ICTD IRLL ANLK EMEE PFEK QQIG SSAM PYKR NPMR SERC CSLA RH
Gene ID:	158
Uniprot:	P30566
Cellular Location:
Calculated MW:	48kDa/54kDa
Observed MW:	55kDa

Synonyms:

ADSL, AMPS, ASASE, ASL

Background:

The protein encoded by this gene belongs to the lyase 1 family. It is an essential enzyme involved in purine metabolism, and catalyzes two non-sequential reactions in the de novo purine biosynthetic pathway: the conversion of succinylaminoimidazole carboxamide ribotide (SAICAR) to aminoimidazole carboxamide ribotide (AICAR) and the conversion of adenylosuccinate (S-AMP) to adenosine monophosphate (AMP). Mutations in this gene are associated with adenylosuccinase deficiency (ADSLD), a disorder marked with psychomotor retardation, epilepsy or autistic features. Alternatively spliced transcript variants have been found for this gene.

UniProt Protein Function:	ADSL: Defects in ADSL are the cause of adenylosuccinase deficiency (ADSL deficiency). ADSL deficiency is an autosomal recessive disorder characterized by the accumulation in the body fluids of succinylaminoimidazole-carboxamide riboside (SAICA-riboside) and succinyladenosine (S-Ado). Most children display marked psychomotor delay, often accompanied by epilepsy or autistic features, or both, although some patients may be less profoundly retarded. Occasionally, growth retardation and muscular wasting are also present. Belongs to the lyase 1 family. Adenylosuccinate lyase subfamily. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:	Protein type:EC 4.3.2.2; Amino Acid Metabolism - alanine, aspartate and glutamate; Nucleotide Metabolism - purine; Lyase Chromosomal Location of Human Ortholog: 22q13.2 Cellular Component: cytosol Molecular Function:adenylosuccinate lyase activity Biological Process: AMP biosynthetic process; purine ribonucleoside monophosphate biosynthetic process; metabolic process; nucleobase, nucleoside and nucleotide metabolic process; purine nucleotide biosynthetic process; protein tetramerization; purine base metabolic process; 'de novo' IMP biosynthetic process Disease: Adenylosuccinase Deficiency
NCBI Summary:	Adenylsuccinate lyase is involved in both de novo synthesis of purines and formation of adenosine monophosphate from inosine monophosphate. It catalyzes two reactions in AMP biosynthesis: the removal of a fumarate from succinylaminoimidazole carboxamide (SAICA) ribotide to give aminoimidazole carboxamide ribotide (AICA) and removal of fumarate from adenylosuccinate to give AMP. Adenylosuccinase deficiency results in succinylpurinemic autism, psychomotor retardation, and , in some cases, growth retardation associated with muscle wasting and epilepsy. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]
UniProt Code:	P30566
NCBI GenInfo Identifier:	6686318
NCBI Gene ID:	158
NCBI Accession:	P30566.2
UniProt Secondary Accession:	P30566,O75495, Q5TI34, B0QY76,
UniProt Related Accession:	P30566
Molecular Weight:	484
NCBI Full Name:	Adenylosuccinate lyase
NCBI Synonym Full Names:	adenylosuccinate lyase
NCBI Official Symbol:	ADSL
NCBI Official Synonym Symbols:	ASL; AMPS; ASASE
NCBI Protein Information:	adenylosuccinate lyase; adenylosuccinase
UniProt Protein Name:	Adenylosuccinate lyase
UniProt Synonym Protein Names:	Adenylosuccinase; ASase
Protein Family:	Adenylosuccinate lyase
UniProt Gene Name:	ADSL
UniProt Entry Name:	PUR8_HUMAN

	Western blot analysis of extracts of various cell lines, using ADSL antibody (CAB6278) 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: 90s.
	Immunofluorescence analysis of HeLa cells using ADSL antibody (CAB6278). Blue: DAPI for nuclear staining.

Antibodies	ELISA Kits
ADSL Antibody (PACO07630)	Human ADSL (Adenylosuccinate lyase) ELISA Kit (HUFI05471)

Secondary Antibody

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