HEXB
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Official Full Name
hexosaminidase B (beta polypeptide) -
Overview
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
Recombinant Proteins
- Mouse
- Rhesus macaque
- Human
- Rat
- HEK293
- Mammalian Cell
- Wheat Germ
- E.coli
- Human Cell
- Mammalian cells
- HEK293T
- In Vitro Cell Free System
- His
- Non
- GST
- His&T7
- His&Fc&Avi
- Myc&DDK
- His&Myc
- Flag
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 |
|---|---|
| Glycosphingolipid biosynthesis - ganglio series | B4GALNT1,ST3GAL5,ST8SIA1,ST6GALNAC6,ST6GALNAC5,ST8SIA5,ST3GAL2,HEXA,ST6GALNAC4,GLB1 |
| Amino sugar and nucleotide sugar metabolism | PMM1,UXS1,GNPNAT1,GMDS,NAGK,CMASA,GALK1,nanA,GFPT1,GMPPB |
| Glycosphingolipid biosynthesis - globo series | B3GALNT1,FUT9,FUT1,FUT9A,GBGT1,GBGT1L4,ST3GAL2,HEXA,ST8SIA1,A4GALT |
| Glycosaminoglycan degradation | HEXA,HYAL4,GNS,HPSE2,GNSA,HYAL2,SGSH,ARSB,SPAM1,HGSNAT |
| Metabolic pathways | ALDH2.1,GUK1,HMGCRB,MGAT4C,ALDOA,CHPT1,CYP19A1,AGPAT9L,GDA,PIGA |
| Other glycan degradation | ENGASE,FUCA1.2,GBA2,HEXA,NEU2,NEU3.1,NEU1,MAN2B1,GBA,NEU3.2 |
| Lysosome | CLN5,ATP6AP1,CTSH,LITAF,GGA2,GALNS,ACP2,ATP6V0A2B,M6PR,ATP6V0D2 |
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 |
|---|---|
| protein heterodimerization activity | YWHAE,ADORA2A,BMP6,UNC13A,PKNOX1,PPARD,MYF5,MCL1A,KCNH1,DVL3 |
| beta-N-acetylhexosaminidase activity | HEXDC,Chil3,HEXA |
| acetylglucosaminyltransferase activity | B3GNTL1,UGGT1,XYLT2,XYLT1,GALNT18A,DPY19L1,OGT,MGAT1A,PIGB,GTDC2 |
| protein homodimerization activity | MFF,ANO2,GSTM3,Defa-rs7,CSF1,ARID3A,PFKM,ATG7,GRPEL1,TRIM37 |
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
Resources
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References
- 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).
