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Target intelligence / Profile preview
Hexosaminidase Subunit Beta (HEXB)
- Target
- HEXB
- Molecular classification
- Enzyme, Glycosyl hydrolase
01
Overview
Hexosaminidase subunit beta (HEXB) is a lysosomal enzyme that plays a crucial role in the degradation of GM2 ganglioside and other complex molecules. It forms a heterodimer with the alpha subunit (HEXA) to create hexosaminidase A and a homodimer to form hexosaminidase B. Mutations in the HEXB gene lead to Sandhoff disease, a severe neurodegenerative disorder caused by the accumulation of GM2 ganglioside in neurons.
- Other names
- Beta-N-acetylhexosaminidase BHex BHEXB_HUMANHexosaminidase B (beta polypeptide)
02
Biological functions
Lysosomal degradationGlycosaminoglycan catabolismSphingolipid catabolism
03
Disease associations
Sandhoff DiseaseGM2 gangliosidosisNeurodegenerative diseaseMotor Neuron Disease
04
Safety considerations
Potential for off-target effects in gene therapiesChallenges in achieving sufficient enzyme activity in the central nervous system
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