GCDH ELISA Kit for Human(Homo sapiens) | EIAab.comGCDH ELISA Kit for Human(Homo sapiens)

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Index > Protein center > GCDH(Gene name) > Human

GCDH (Gene name),

Glutaryl-CoA dehydrogenase, mitochondrial (Protein name ), GCDH_HUMAN from NCBI database.

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General Annotation

Antigen Annotation

Predicted Eptitope

Vaild Sequence

Related Databases

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Gene name:

GCDH;

Protein name:

Glutaryl-CoA dehydrogenase, mitochondrial(GCD);

Alternative:

Organism:

Human (Homo sapiens).

General Annotation

Sub Unit:

Homotetramer.

Function:

Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive.

Subcellular Location:

Mitochondrion matrix

Protein Attributes:

Sequence length:

438

Sequence:

50:

100:

150:

200:

250:

300:

350:

400:

438:

IRHAMNLEAV | NTYEGTHDIH | ALILGRAITG | IQAFTASK

3D Structure:

N/A

Predicted Eptitope:

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Vaild Sequence:

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Related Databases

STRING:

9606.ENSP00000222214

String:

Q92947

MIM:

231670

UniGene:

Hs.532699

KEGG:

hsa:2639

Pfam:

PF00441

SMR:

Q92947

Uniprot:

Q92947

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ELISA Kit for Human GCD

Cat.:

E10449h

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96T

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ELISA Kit for Human GCD

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E10449p

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96T

ELISA Kit for Human GCD

Cat.:

E10449m

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96T

ELISA Kit for Human GCD

Cat.:

E10449b

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96T

CLIA Kit for Human GCD

Cat.:

U10449p

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Packing:

96T

CLIA Kit for Human GCD

Cat.:

U10449m

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MSDS:

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Packing:

96T

CLIA Kit for Human GCD

Cat.:

U10449h

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Packing:

96T

CLIA Kit for Human GCD

Cat.:

U10449b

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96T

Polyclonal Antibody for Human GCD

Cat.:

P10449Rb-h

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Packing:

40ug/0.2ml

Polyclonal Antibody for Human GCD

Cat.:

P10449Rb-m

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Packing:

40ug/0.2ml

Monoclonal Antibody for Human GCD

Protein for Human GCD

R&D Technical Data

For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.

Precision

For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.

Recovery

For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.

Linearity

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References

1.

"Pork and human cDNAs encoding glutaryl-CoA dehydrogenase."
Goodman S.I. , Kratz L.E. , Frerman F.E.
Prog. Clin. Biol. Res.375:169-173(1992) [PubMed] [Europe PMC] [Abstract]

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM LONG)
tissue: Liver.
2.

"Cloning of glutaryl-CoA dehydrogenase cDNA, and expression of wild type and mutant enzymes in Escherichia coli."
Goodman S.I. , Kratz L.E. , Digiulio K.A. , Biery B.J. , Goodman K.E. , Isaya G. , Frerman F.E.
Hum. Mol. Genet.4:1493-1498(1995) [PubMed] [Europe PMC] [Abstract]

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORMS LONG AND SHORT);FUNCTION;CATALYTIC ACTIVITY;VARIANT GA1 HIS-295
tissue: Liver.
3.

"The human glutaryl-CoA dehydrogenase gene: report of intronic sequences and of 13 novel mutations causing glutaric aciduria type I."
Schwartz M. , Christensen E. , Superti-Furga A. , Brandt N.J.
Hum. Genet.102:452-458(1998) [PubMed] [Europe PMC] [Abstract]

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA];VARIANTS GA1
4.

"Cloning of human full-length CDSs in BD Creator(TM) system donor vector."
Kalnine N. , Chen X. , Rolfs A. , Halleck A. , Hines L. , Eisenstein S. , Koundinya M. , Raphael J. , Moreira D. , Kelley T. , LaBaer J. , Lin Y. , Phelan M. , Farmer A.
Submitted (2003-05) to the EMBL/GenBank/DDBJ databases

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM LONG)
5.

"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Ota T. , Suzuki Y. , Nishikawa T. , Otsuki T. , Sugiyama T. , Irie R. , Wakamatsu A. , Hayashi K. , Sato H. , Nagai K. , Kimura K. , Makita H. , Sekine M. , Obayashi M. , Nishi T. , Shibahara T. , Tanaka T. , Ishii S. , more...
Nat. Genet.36:40-45(2004) [PubMed] [Europe PMC] [Abstract]

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM LONG)
tissue: Umbilical cord blood.
6.

"The DNA sequence and biology of human chromosome 19."
Grimwood J. , Gordon L.A. , Olsen A.S. , Terry A. , Schmutz J. , Lamerdin J.E. , Hellsten U. , Goodstein D. , Couronne O. , Tran-Gyamfi M. , Aerts A. , Altherr M. , Ashworth L. , Bajorek E. , Black S. , Branscomb E. , Caenepeel S. , Carrano A.V. , more...
Nature428:529-535(2004) [PubMed] [Europe PMC] [Abstract]

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA]
7.

Mural R.J. , Istrail S. , Sutton G.G. , Florea L. , Halpern A.L. , Mobarry C.M. , Lippert R. , Walenz B. , Shatkay H. , Dew I. , Miller J.R. , Flanigan M.J. , Edwards N.J. , Bolanos R. , Fasulo D. , Halldorsson B.V. , Hannenhalli S. , Turner R. , more...
Submitted (2005-07) to the EMBL/GenBank/DDBJ databases

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA]
8.

"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
The MGC Project Team
Genome Res.14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM LONG)
tissue: Lymph.
9.

"Specific glutaryl-CoA dehydrogenating activity is deficient in cultured fibroblasts from glutaric aciduria patients."
Hyman D.B. , Tanaka K.
J. Clin. Invest.73:778-784(1984) [PubMed] [Europe PMC] [Abstract]

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: FUNCTION;ENZYME REGULATION;BIOPHYSICOCHEMICAL PROPERTIES
10.

"Glutaryl-CoA dehydrogenase mutations in glutaric acidemia (type I): review and report of thirty novel mutations."
Goodman S.I. , Stein D.E. , Schlesinger S. , Christensen E. , Schwartz M. , Greenberg C.R. , Elpeleg O.N.
Hum. Mutat.12:141-144(1998) [PubMed] [Europe PMC] [Abstract]

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: REVIEW ON VARIANTS
11.

"Kinetic mechanism of glutaryl-CoA dehydrogenase."
Rao K.S. , Albro M. , Dwyer T.M. , Frerman F.E.
Biochemistry45:15853-15861(2006) [PubMed] [Europe PMC] [Abstract]

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: FUNCTION;BIOPHYSICOCHEMICAL PROPERTIES
12.

"Initial characterization of the human central proteome."
Burkard T.R. , Planyavsky M. , Kaupe I. , Breitwieser F.P. , Buerckstuemmer T. , Bennett K.L. , Superti-Furga G. , Colinge J.
BMC Syst. Biol.5:17-17(2011) [PubMed] [Europe PMC] [Abstract]

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS]
13.

"Crystal structures of human glutaryl-CoA dehydrogenase with and without an alternate substrate: structural bases of dehydrogenation and decarboxylation reactions."
Fu Z. , Wang M. , Paschke R. , Rao K.S. , Frerman F.E. , Kim J.-J.P.
Biochemistry43:9674-9684(2004) [PubMed] [Europe PMC] [Abstract]

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: X-RAY CRYSTALLOGRAPHY (2.1 ANGSTROMS) OF 47-438 IN COMPLEXES WITH SUBSTRATE ANALOG AND FAD;SUBUNIT
14.

"The effect of a Glu370Asp mutation in glutaryl-CoA dehydrogenase on proton transfer to the dienolate intermediate."
Rao K.S. , Fu Z. , Albro M. , Narayanan B. , Baddam S. , Lee H.-J.K. , Kim J.-J.P. , Frerman F.E.
Biochemistry46:14468-14477(2007) [PubMed] [Europe PMC] [Abstract]

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: X-RAY CRYSTALLOGRAPHY (2.3 ANGSTROMS) OF 45-438 OF MUTANT ASP-414 IN COMPLEXES WITH SUBSTRATE AND FAD
15.

"Gene structure and mutations of glutaryl-coenzyme A dehydrogenase: impaired association of enzyme subunits that is due to an A421V substitution causes glutaric acidemia type I in the Amish."
Biery B.J. , Stein D.E. , Morton D.H. , Goodman S.I.
Am. J. Hum. Genet.59:1006-1011(1996) [PubMed] [Europe PMC] [Abstract]

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANTS GA1
16.

"Glutaric aciduria type I in the Arab and Jewish communities in Israel."
Anikster Y. , Shaag A. , Joseph A. , Mandel H. , Ben-Zeev B. , Christensen E. , Elpeleg O.N.
Am. J. Hum. Genet.59:1012-1018(1996) [PubMed] [Europe PMC] [Abstract]

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANTS GA1 ARG-101; PRO-283; THR-293; LEU-305; ARG-390 AND ILE-416
17.

"Severe phenotype despite high residual glutaryl-CoA dehydrogenase activity: a novel mutation in a Turkish patient with glutaric aciduria type I."
Muehlhausen C. , Christensen E. , Schwartz M. , Muschol N. , Ullrich K. , Lukacs Z.
J. Inherit. Metab. Dis.26:713-714(2003) [PubMed] [Europe PMC] [Abstract]

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANT GA1 VAL-263
18.

"Disease-causing missense mutations affect enzymatic activity, stability and oligomerization of glutaryl-CoA dehydrogenase (GCDH)."
Keyser B. , Muehlhausen C. , Dickmanns A. , Christensen E. , Muschol N. , Ullrich K. , Braulke T.
Hum. Mol. Genet.17:3854-3863(2008) [PubMed] [Europe PMC] [Abstract]

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: CHARACTERIZATION OF VARIANTS GA1 GLY-138; TRP-402 AND LYS-414;SUBUNIT
19.

"Molecular analysis of Cypriot patients with Glutaric aciduria type I: Identification of two novel mutations."
Georgiou T. , Nicolaidou P. , Hadjichristou A. , Ioannou R. , Dionysiou M. , Siama E. , Chappa G. , Anastasiadou V. , Drousiotou A.
Clin. Biochem.47:1300-1305(2014) [PubMed] [Europe PMC] [Abstract]

[15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANTS GA1 ASP-64; VAL-268; ARG-375; TRP-402 AND MET-429

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