hexb
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  • Official Full Name
  • hexosaminidase B (beta polypeptide)
  • Background
  • Hexosaminidase B is the beta subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines.Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes.Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases.Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses.Beta subunit gene mutations lead to Sandhoff disease (GM2-gangliosidosis type II).
  • Synonyms
  • HEXB; hexosaminidase B (beta polypeptide); beta-hexosaminidase subunit beta; Beta hexosaminidase beta chain; Beta N acetylhexosaminidase; Beta-hexosaminidase subunit beta chain A; Beta-N-acetylhexosaminidase subunit beta; Cervical cancer proto oncogene 7 protein; Cervical cancer proto-oncogene 7 protein; ENC 1AS; HCC 7; HCC-7; HCC7; HEX B; HEXB_HUMAN; Hexosaminidase B; Hexosaminidase subunit B; HexosaminidaseB; N acetyl beta glucosaminidase; N-acetyl-beta-glucosaminidase subunit beta; ENC-1AS
Cat.#:HEXB-1090HTag:Myc|DDK
Source (Host):HEK293Species:Human
Product nameRecombinant Human HEXB Protein, MYC/DDK-tagged
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Cat.#:HEXB-4711HTag:GST
Source (Host):Wheat GermSpecies:Human
Product nameRecombinant Human HEXB Protein, GST-tagged
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Cat.#:HEXB-27728THTag:N/A
Source (Host):Wheat germSpecies:Human
Product nameRecombinant Human HEXB
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Cat.#:HEXB-302HTag:His
Source (Host):HEK293Species:Human
Product nameRecombinant Human HEXB, His-tagged
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Cat.#:HEXB-644HTag:His
Source (Host):E. coliSpecies:Human
Product nameRecombinant Human HEXB protein, His-tagged
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Cat.#:HEXB-645HTag:His
Source (Host):E. coliSpecies:Human
Product nameRecombinant Human HEXB protein, His-tagged
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Cat.#:Hexb-1014MTag:His
Source (Host):HEK293Species:Mouse
Product nameRecombinant Mouse Hexb Protein, His-tagged
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Cat.#:Hexb-646MTag:His
Source (Host):E. coliSpecies:Mouse
Product nameRecombinant Mouse Hexb protein, His-tagged
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Cat.#:Hexb-647MTag:His
Source (Host):E. coliSpecies:Mouse
Product nameRecombinant Mouse Hexb protein, His-tagged
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Cat.#:HEXB-7594MTag:His
Source (Host):Mammalian CellsSpecies:Mouse
Product nameRecombinant Mouse HEXB Protein
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Cat.#:HEXB-2834RTag:His
Source (Host):Mammalian CellsSpecies:Rat
Product nameRecombinant Rat HEXB Protein
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Cat.#:Hexb-648RTag:His|T7
Source (Host):E. coliSpecies:Rat
Product nameRecombinant Rat Hexb protein, His & T7-tagged
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Cat.#:HEXB-2070RTag:His
Source (Host):Mammalian CellsSpecies:Rhesus Macaque
Product nameRecombinant Rhesus monkey HEXB Protein, His-tagged
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Cat.#:HEXB-756HCLTag:N/A
Source (Host):HEK293Species:Human
Product nameRecombinant Human HEXB cell lysate
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Involved Pathway

HEXB involved in several pathways and played different roles in them. We selected most pathways HEXB participated on our site, such as Other glycan degradation, Amino sugar and nucleotide sugar metabolism, Glycosaminoglycan degradation, which may be useful for your reference. Also, other proteins which involved in the same pathway with HEXB were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.
Pathway Name
Pathway Related Protein
Other glycan degradation
GLB1; MAN2B2; FUCA1.2; NEU4; NEU3.1; HEXA; FUCA1; NEU1; NEU3.4; GBA
Amino sugar and nucleotide sugar metabolism
PMM2; GNPDA2; GPI; UAP1L1; CYB5R2; GPIA; UGDH; UGP2A; HK3; UXS1
Glycosaminoglycan degradation
GNSB; SGSH; GALNS; HYAL1; HEXB; HYAL2; HPSE; HGSNAT; NAGLU; GUSB
Glycosphingolipid biosynthesis - globo series
A4GALT; HEXB; FUT9; GBGT1L4; ST3GAL1; GLA; GBGT1; FUT2; NAGA; FUT1
Glycosphingolipid biosynthesis - ganglio series
SLC33A1; ST6GALNAC3; HEXB; B3GALT4; ST3GAL5; ST6GALNAC4; ST3GAL2; ST6GALNAC6; GLB1; ST8SIA1
Metabolic pathways
CKMA; EPT1; TDO2; PIGV; UPP1; NDUFC2-KCTD14; RDH8; FPGT; PCK2; MGAT1
Lysosome
ATP6V0A2; ATP6V0A4; CLTCA; DNASE2; CLTCB; GLA; ACP2; ATP6V0A1; DNASE2B; GGA1

Protein Function

HEXB has several biochemical functions, for example, acetylglucosaminyltransferase activity, beta-N-acetylhexosaminidase activity, protein heterodimerization activity. Some of the functions are cooperated with other proteins, some of the functions could acted by HEXB itself. We selected most functions HEXB had, and list some proteins which have the same functions with HEXB. You can find most of the proteins on our site.
Function
Related Protein
Function acetylglucosaminyltransferase activity
Related Protein EXT1; HEXB; LARGE; MGAT1A; B3GNTL1; GALNT18A; DPY19L1; GALNTL4; DPY19L4; XYLT2
Function beta-N-acetylhexosaminidase activity
Related Protein HEXA; HEXB; HEXDC; Chil3
Function protein heterodimerization activity
Related Protein HIST1H2AG; HIST1H2AK; SYT14; SAE1; SEPHS1; ADD2; PANX2; CA; DDIT3; H2-AA
Function protein homodimerization activity
Related Protein TP53I3; BCL2A1; GRIK1; FGFR1OP; CBLN1; CAMK2B; BAXB; SUMF1; ABCG2; SPPL3

Interacting Protein

HEXB has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with HEXB here. Most of them are supplied by our site. Hope this information will be useful for your research of HEXB.
CSNK2B; SOD1; Tuba3a; Bach1; Nup214; Tgs1; SMC6; TSNAX; Kif19

HEXB Related Articles

Villamizar-Schiller, IT; Pabon, LA; et al. Neurological and cardiac responses after treatment with miglustat and a ketogenic diet in a patient with Sandhoff disease. EUROPEAN JOURNAL OF MEDICAL GENETICS 58:180-183(2015).
Walia, JS; Altaleb, N; et al. Long-Term Correction of Sandhoff Disease Following Intravenous Delivery of rAAV9 to Mouse Neonates. MOLECULAR THERAPY 23:414-422(2015).