HEXA

This gene encodes a member of the glycosyl hydrolase 20 family of proteins. The encoded preproprotein is proteolytically processed to generate the alpha subunit of the lysosomal enzyme beta-hexosaminidase. This enzyme, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mutations in this gene lead to an accumulation of GM2 ganglioside in neurons, the underlying cause of neurodegenerative disorders termed the GM2 gangliosidoses, including Tay-Sachs disease (GM2-gangliosidosis type I). Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed. [provided by RefSeq, Jan 2016]
Full Name
Hexosaminidase Subunit Alpha
Function
Hydrolyzes the non-reducing end N-acetyl-D-hexosamine and/or sulfated N-acetyl-D-hexosamine of glycoconjugates, such as the oligosaccharide moieties from proteins and neutral glycolipids, or from certain mucopolysaccharides (PubMed:11707436, PubMed:9694901, PubMed:8672428, PubMed:8123671).

The isozyme S is as active as the isozyme A on the anionic bis-sulfated glycans, the chondroitin-6-sulfate trisaccharide (C6S-3), and the dermatan sulfate pentasaccharide, and the sulfated glycosphingolipid SM2 (PubMed:11707436).

The isozyme B does not hydrolyze each of these substrates, however hydrolyzes efficiently neutral oligosaccharide (PubMed:11707436).

Only the isozyme A is responsible for the degradation of GM2 gangliosides in the presence of GM2A (PubMed:9694901, PubMed:8672428, PubMed:8123671).
Biological Process
Carbohydrate metabolic process Source: InterPro
Ganglioside catabolic process Source: UniProtKB
Glycosaminoglycan biosynthetic process Source: UniProtKB
Glycosaminoglycan metabolic process Source: ComplexPortal
Cellular Location
Lysosome
Involvement in disease
GM2-gangliosidosis 1 (GM2G1):
An autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. It is characterized by GM2 gangliosides accumulation in the absence of HEXA activity, leading to neurodegeneration and, in the infantile form, death in early childhood. It exists in several forms: infantile (most common and most severe), juvenile and adult (late-onset).
PTM
N-linked glycan at Asn-115 consists of Man3-GlcNAc2 (PubMed:1533633, PubMed:16698036, PubMed:19159218) (Probable). N-linked glycan at Asn-157 consists of either GlcNAc or GlcNAc2-Man7-9. N-linked glycan at Asn-295 consists of either GlcNAc, GlcNAc-Fuc, or GlcNAc2-Man4 (Probable).
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Anti-HEXA antibodies

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Mouse Anti-HEXA Recombinant Antibody (CF200) (CBMAB-FT290LY)

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Target: HEXA
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: CF200
Application*: ELISA, WB
Datasheet SDS

Mouse Anti-HEXA Recombinant Antibody (CBT3891) (V2LY-0625-LY2686)

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Target: HEXA
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: CBT3891
Application*: WB, F
Datasheet SDS

Mouse Anti-HEXA Recombinant Antibody (A160) (CBMAB-AP9589LY)

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Target: HEXA
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: A160
Application*: ELISA, WB
Datasheet SDS

Mouse Anti-HEXA Recombinant Antibody (A161) (CBMAB-AP2973LY)

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Target: HEXA
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: A161
Application*: Dot blot, ELISA, WB
Datasheet SDS

Mouse Anti-HEXA Recombinant Antibody (CBYJT-1363) (CBMAB-T0385-YJ)

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Target: HEXA
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBYJT-1363
Application*: WB, IC, P, C, E
Datasheet SDS

Mouse Anti-HEXA Recombinant Antibody (CBFYH-0977) (CBMAB-H1916-FY)

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Target: HEXA
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: CBFYH-0977
Application*: E, F, WB
Datasheet SDS

Mouse Anti-HEXA (AA 1-529) Recombinant Antibody (CBFYH-0974) (CBMAB-H1914-FY)

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Target: HEXA
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: CBFYH-0974
Application*: IH, WB
Datasheet SDS

Mouse Anti-HEXA Recombinant Antibody (CBFYH-0973) (CBMAB-H1913-FY)

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Target: HEXA
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: CBFYH-0973
Application*: E, WB
Datasheet SDS

Mouse Anti-HEXA (AA 1-529) Recombinant Antibody (CBFYH-0972) (CBMAB-H1912-FY)

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Target: HEXA
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: CBFYH-0972
Application*: E, WB
Datasheet SDS

Mouse Anti-HEXA (AA 1-529) Recombinant Antibody (CBFYH-0971) (CBMAB-H1911-FY)

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Target: HEXA
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: CBFYH-0971
Application*: P, WB
Datasheet SDS

Mouse Anti-HEXA Monoclonal Antibody (CBFYH-3053) (CBMAB-H1909-FY)

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Target: HEXA
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: CBFYH-3053
Application*: F
Datasheet SDS

Mouse Anti-HEXA (AA 89-529) Recombinant Antibody (CBFYH-0976) (CBMAB-H0412-FY)

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Target: HEXA
Host: Mouse
Antibody Isotype: IgG2a, λ
Specificity: Human
Clone: CBFYH-0976
Application*: WB, F, E
Datasheet SDS
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(P): Predicted
* Abbreviations
  • AActivation
  • AGAgonist
  • APApoptosis
  • BBlocking
  • BABioassay
  • BIBioimaging
  • CImmunohistochemistry-Frozen Sections
  • CIChromatin Immunoprecipitation
  • CTCytotoxicity
  • CSCostimulation
  • DDepletion
  • DBDot Blot
  • EELISA
  • ECELISA(Cap)
  • EDELISA(Det)
  • ESELISpot
  • EMElectron Microscopy
  • FFlow Cytometry
  • FNFunction Assay
  • GSGel Supershift
  • IInhibition
  • IAEnzyme Immunoassay
  • ICImmunocytochemistry
  • IDImmunodiffusion
  • IEImmunoelectrophoresis
  • IFImmunofluorescence
  • IHImmunohistochemistry
  • IMImmunomicroscopy
  • IOImmunoassay
  • IPImmunoprecipitation
  • ISIntracellular Staining for Flow Cytometry
  • LALuminex Assay
  • LFLateral Flow Immunoassay
  • MMicroarray
  • MCMass Cytometry/CyTOF
  • MDMeDIP
  • MSElectrophoretic Mobility Shift Assay
  • NNeutralization
  • PImmunohistologyp-Paraffin Sections
  • PAPeptide Array
  • PEPeptide ELISA
  • PLProximity Ligation Assay
  • RRadioimmunoassay
  • SStimulation
  • SESandwich ELISA
  • SHIn situ hybridization
  • TCTissue Culture
  • WBWestern Blot
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