HEXB CLIA Kit for Human(Homo sapiens) | EIAab.comHEXB CLIA Kit for Human(Homo sapiens)

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HEXB (Gene name),

Beta-hexosaminidase subunit beta (Protein name ), HEXB_HUMAN from NCBI database.

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General Annotation

Antigen Annotation

Predicted Eptitope

Vaild Sequence

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Gene name:

HEXB(HCC7);

Protein name:

Beta-hexosaminidase subunit beta;

Alternative:

Cervical cancer proto-oncogene 7 protein(HCC-7);Beta-N-acetylhexosaminidase subunit beta(Hexosaminidase subunit B);N-acetyl-beta-glucosaminidase subunit beta;

Organism:

Human (Homo sapiens).

General Annotation

Sub Unit:

There are 3 forms of beta-hexosaminidase: hexosaminidase A is a trimer composed of one subunit alpha, one subunit beta chain A and one subunit beta chain B; hexosaminidase B is a tetramer of two subunit beta chains A and two subunit beta chains B; hexosaminidase S is an homodimer of two alpha subunits. The two beta chains are derived from the cleavage of the beta subunit.

Function:

Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues.

Subcellular Location:

Lysosome

Protein Attributes:

Sequence length:

556

Sequence:

50:

100:

150:

200:

250:

300:

350:

400:

450:

500:

550:

556:

CNHENM

3D Structure:

N/A

Predicted Eptitope:

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Vaild Sequence:

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Related Databases

STRING:

9606.ENSP00000261416

SMR:

P07686

KEGG:

hsa:3074

UniGene:

Hs.69293

MIM:

268800

String:

P07686

Pfam:

PF00728

Uniprot:

P07686

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ELISA Kit for Human Beta-hexosaminidase subunit beta

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E8893r

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96T

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ELISA Kit for Human Beta-hexosaminidase subunit beta

Cat.:

E8893h

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96T

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ELISA Kit for Human Beta-hexosaminidase subunit beta

Cat.:

E8893m

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96T

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ELISA Kit for Human Beta-hexosaminidase subunit beta

Cat.:

E8893p

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Packing:

96T

CLIA Kit for Human Beta-hexosaminidase subunit beta

Cat.:

U8893h

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Looking for manual.

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96T

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CLIA Kit for Human Beta-hexosaminidase subunit beta

Cat.:

U8893m

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MSDS:

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Packing:

96T

CLIA Kit for Human Beta-hexosaminidase subunit beta

Cat.:

U8893r

Price:

Please sign in first.

MSDS:

Please sign in first.

Packing:

96T

CLIA Kit for Human Beta-hexosaminidase subunit beta

Cat.:

U8893p

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Packing:

96T

Polyclonal Antibody for Human Beta-hexosaminidase subunit beta

Cat.:

P8893Rb-h

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40ug/0.2ml

Polyclonal Antibody for Human Beta-hexosaminidase subunit beta

Cat.:

P8893Rb-m

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Packing:

40ug/0.2ml

Polyclonal Antibody for Human Beta-hexosaminidase subunit beta

Monoclonal Antibody for Human Beta-hexosaminidase subunit beta

Protein for Human Beta-hexosaminidase subunit beta

R&D Technical Data

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

Precision

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Recovery

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Linearity

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References

1.

"Isolation of cDNA clones coding for the alpha-subunit of human beta-hexosaminidase. Extensive homology between the alpha- and beta-subunits and studies on Tay-Sachs disease."
Korneluk R.G. , Mahuran D.J. , Neote K. , Klavins M.H. , O'Dowd B.F. , Tropak M. , Willard H.F. , Anderson M.-J. , Lowden J.A. , Gravel R.A.
J. Biol. Chem.261:8407-8413(1986) [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]
2.

"Characterization of the human HEXB gene encoding lysosomal beta-hexosaminidase."
Neote K. , Bapat B. , Dumbrille-Ross A. , Troxel C. , Schuster S.M. , Mahuran D.J. , Gravel R.A.
Genomics3:279-286(1988) [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]
3.

"Gene encoding the human beta-hexosaminidase beta chain: extensive homology of intron placement in the alpha- and beta-chain genes."
Proia R.L.
Proc. Natl. Acad. Sci. U.S.A.85:1883-1887(1988) [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]
4.

"Identification of a new proto-oncogene in human cancers."
Kim J.W.
Submitted (2001-05) 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]
5.

"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 (2003-08) 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]
6.

"The DNA sequence and comparative analysis of human chromosome 5."
Schmutz J. , Martin J. , Terry A. , Couronne O. , Grimwood J. , Lowry S. , Gordon L.A. , Scott D. , Xie G. , Huang W. , Hellsten U. , Tran-Gyamfi M. , She X. , Prabhakar S. , Aerts A. , Altherr M. , Bajorek E. , Black S. , more...
Nature431:268-274(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.

"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]
tissue: Skin.
8.

"Synthesis and assembly of a catalytically active lysosomal enzyme, beta-hexosaminidase B, in a cell-free system."
Sonderfeld-Fresko S. , Proia R.L.
J. Biol. Chem.263:13463-13469(1988) [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] OF 1-67
9.

"Translation initiation in the HEXB gene encoding the beta-subunit of human beta-hexosaminidase."
Neote K. , Brown C.A. , Mahuran D.J. , Gravel R.A.
J. Biol. Chem.265:20799-20806(1990) [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] OF 1-52
10.

"Localization of the pro-sequence within the total deduced primary structure of human beta-hexosaminidase B."
Stirling J. , Leung A. , Gravel R.A. , Mahuran D.
FEBS Lett.231:47-50(1988) [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: PROTEIN SEQUENCE OF 43-57 AND 122-151
11.

"Characterization of human placental beta-hexosaminidase I2. Proteolytic processing intermediates of hexosaminidase A."
Mahuran D.J.
J. Biol. Chem.265:6794-6799(1990) [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: PROTEIN SEQUENCE OF 45-54 AND 315-324
12.

"The amino-terminal sequences in the pro-alpha and -beta polypeptides of human lysosomal beta-hexosaminidase A and B are retained in the mature isozymes."
Hubbes M. , Callahan J. , Gravel R. , Mahuran D.
FEBS Lett.249:316-320(1989) [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: PROTEIN SEQUENCE OF 50-59
13.

"Proteolytic processing of pro-alpha and pro-beta precursors from human beta-hexosaminidase. Generation of the mature alpha and beta a beta b subunits."
Mahuran D.J. , Neote K. , Klavins M.H. , Leung A. , Gravel R.A.
J. Biol. Chem.263:4612-4618(1988) [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: PROTEIN SEQUENCE OF 122-151 AND 315-340
14.

"Isolation of cDNA clones coding for the beta subunit of human beta-hexosaminidase."
O'Dowd B.F. , Quan F. , Willard H.F. , Lamhonwah A.-M. , Korneluk R.G. , Lowden J.A. , Gravel R.A. , Mahuran D.J.
Proc. Natl. Acad. Sci. U.S.A.82:1184-1188(1985) [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] OF 226-283
15.

"Oligosaccharide structure and amino acid sequence of the major glycopeptides of mature human beta-hexosaminidase."
O'Dowd B.F. , Cumming D.A. , Gravel R.A. , Mahuran D.J.
Biochemistry27:5216-5226(1988) [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: STRUCTURE OF CARBOHYDRATES
16.

"Identification and quantification of N-linked glycoproteins using hydrazide chemistry, stable isotope labeling and mass spectrometry."
Zhang H. , Li X.-J. , Martin D.B. , Aebersold R.
Nat. Biotechnol.21:660-666(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: GLYCOSYLATION AT ASN-327
17.

"Complete analysis of the glycosylation and disulfide bond pattern of human beta-hexosaminidase B by MALDI-MS."
Schuette C.G. , Weisgerber J. , Sandhoff K.
Glycobiology11:549-556(2001) [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: DISULFIDE BONDS;GLYCOSYLATION AT ASN-84; ASN-142; ASN-190 AND ASN-327
18.

"Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry."
Chen R. , Jiang X. , Sun D. , Han G. , Wang F. , Ye M. , Wang L. , Zou H.
J. Proteome Res.8:651-661(2009) [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: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-84; ASN-323 AND ASN-327
tissue: Liver.
19.

"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]
20.

"Bacterial chitobiase structure provides insight into catalytic mechanism and the basis of Tay-Sachs disease."
Tews I. , Perrakis A. , Oppenheim A. , Dauter Z. , Wilson K.S. , Vorgias C.E.
Nat. Struct. Biol.3:638-648(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: 3D-STRUCTURE MODELING
21.

"Crystal structure of human beta-hexosaminidase B: understanding the molecular basis of Sandhoff and Tay-Sachs disease."
Mark B.L. , Mahuran D.J. , Cherney M.M. , Zhao D. , Knapp S. , James M.N.
J. Mol. Biol.327:1093-1109(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: X-RAY CRYSTALLOGRAPHY (2.4 ANGSTROMS) OF 50-556;DISULFIDE BONDS
22.

"The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease."
Maier T. , Strater N. , Schuette C.G. , Klingenstein R. , Sandhoff K. , Saenger W.
J. Mol. Biol.328:669-681(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: X-RAY CRYSTALLOGRAPHY (2.25 ANGSTROMS) OF 42-556;DISULFIDE BONDS
23.

"The biochemistry of HEXA and HEXB gene mutations causing GM2 gangliosidosis."
Mahuran D.J.
Biochim. Biophys. Acta1096:87-94(1991) [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
24.

"Molecular basis of an adult form of beta-hexosaminidase B deficiency with motor neuron disease."
Banerjee P. , Siciliano L. , Oliveri D. , McCabe N.R. , Boyers M.J. , Horwitz A.L. , Li S.-C. , Dawson G.
Biochem. Biophys. Res. Commun.181:108-115(1991) [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 GM2G2 SER-456;VARIANT VAL-207
25.

"A novel exon mutation in the human beta-hexosaminidase beta subunit gene affects 3' splice site selection."
Wakamatsu N. , Kobayashi H. , Miyatake T. , Tsuji S.
J. Biol. Chem.267:2406-2413(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: VARIANT GM2G2 LEU-417
26.

"Molecular basis of an adult form of Sandhoff disease: substitution of glutamine for arginine at position 505 of the beta-chain of beta-hexosaminidase results in a labile enzyme."
Bolhuis P.A. , Ponne N.J. , Bikker H. , Baas F. , Vianney de Jong J.M.B.
Biochim. Biophys. Acta1182:142-146(1993) [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 GM2G2 GLN-505
27.

"A novel missense mutation (C522Y) is present in the beta-hexosaminidase beta-subunit gene of a Japanese patient with infantile Sandhoff disease."
Kuroki Y. , Itoh K. , Nadaoka Y. , Tanaka T. , Sakuraba H.
Biochem. Biophys. Res. Commun.212:564-571(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: VARIANT GM2G2 TYR-534
28.

"A common beta hexosaminidase gene mutation in adult Sandhoff disease patients."
Gomez-Lira M. , Sangalli A. , Mottes M. , Perusi C. , Pignatti P.F. , Rizzuto N. , Salviati A.
Hum. Genet.96:417-422(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: VARIANTS GM2G2 TYR-309 AND LEU-417
29.

"A second, large deletion in the HEXB gene in a patient with infantile Sandhoff disease."
Zhang Z.-X. , Wakamatsu N. , Akerman B.R. , Mules E.H. , Thomas G.H. , Gravel R.A.
Hum. Mol. Genet.4:777-780(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: VARIANT GM2G2 LEU-62
30.

"Significance of two point mutations present in each HEXB allele of patients with adult GM2 gangliosidosis (Sandhoff disease) homozygosity for the Ile207-->Val substitution is not associated with a clinical or biochemical phenotype."
Redonnet-Vernhet I. , Mahuran D.J. , Salvayre R. , Dubas F. , Levade T.
Biochim. Biophys. Acta1317:127-133(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: VARIANT GM2G2 GLN-505;VARIANT VAL-207
31.

"Molecular basis of heat labile hexosaminidase B among Jews and Arabs."
Narkis G. , Adam A. , Jaber L. , Pennybacker M. , Proia R.L. , Navon R.
Hum. Mutat.10:424-429(1997) [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 GM2G2 THR-543
32.

"Two mutations remote from an exon/intron junction in the beta-hexosaminidase beta-subunit gene affect 3'-splice site selection and cause Sandhoff disease."
Fujimaru M. , Tanaka A. , Choeh K. , Wakamatsu N. , Sakuraba H. , Isshiki G.
Hum. Genet.103:462-469(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: VARIANT GM2G2 ARG-255
33.

"A Pro504 --> Ser substitution in the beta-subunit of beta-hexosaminidase A inhibits alpha-subunit hydrolysis of GM2 ganglioside, resulting in chronic Sandhoff disease."
Hou Y. , McInnes B. , Hinek A. , Karpati G. , Mahuran D.
J. Biol. Chem.273:21386-21392(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: VARIANT GM2G2 SER-504

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