TY - CHAP
T1 - Gangliosides in Neurodegenerative Diseases
AU - Ledeen, Robert
AU - Chowdhury, Suman
N1 - Publisher Copyright:
© 2023, The Author(s), under exclusive license to Springer Nature Switzerland AG.
PY - 2023
Y1 - 2023
N2 - The main purpose of this chapter is to summarize the chief findings on ganglioside changes/interactions with some of the neurodegenerative disorders. For the latter we have focused on three diseases that have seen especially intensive study in that regard: Parkinson’s, Alzheimer’s, and Huntington’s diseases. Parkinson’s disease (PD) has received the most intensive study with revelation of systemic deficiency of GM1 in brain and all peripheral tissues that have been analyzed to date; this pointed to GM1 replacement as a promising therapy which proved only partially successful when tried for reasons that are discussed. Huntington’s disease resembles PD in also manifesting GM1 deficiency, which did, however, respond to GM1 replacement therapy – apparently due to GM1 being administered directly into the brain. Alzheimer’s disease was more complex in relation to gangliosides, with b-series (GD1b, GT1b) apparently depressed along with a-series. GM1 administered in brain appeared to induce improvement, but in a limited number of patients. We summarize studies showing why GM1 is of critical importance in neuronal function, and we also briefly point to a few additional neurological disorders in which one or more ganglioside changes have been implicated.
AB - The main purpose of this chapter is to summarize the chief findings on ganglioside changes/interactions with some of the neurodegenerative disorders. For the latter we have focused on three diseases that have seen especially intensive study in that regard: Parkinson’s, Alzheimer’s, and Huntington’s diseases. Parkinson’s disease (PD) has received the most intensive study with revelation of systemic deficiency of GM1 in brain and all peripheral tissues that have been analyzed to date; this pointed to GM1 replacement as a promising therapy which proved only partially successful when tried for reasons that are discussed. Huntington’s disease resembles PD in also manifesting GM1 deficiency, which did, however, respond to GM1 replacement therapy – apparently due to GM1 being administered directly into the brain. Alzheimer’s disease was more complex in relation to gangliosides, with b-series (GD1b, GT1b) apparently depressed along with a-series. GM1 administered in brain appeared to induce improvement, but in a limited number of patients. We summarize studies showing why GM1 is of critical importance in neuronal function, and we also briefly point to a few additional neurological disorders in which one or more ganglioside changes have been implicated.
KW - Alzheimer’s disease
KW - GM1 essentiality
KW - Gangliosides
KW - Huntington’s disease
KW - Neurodegenerative disorders
KW - Parkinson’s disease
UR - http://www.scopus.com/inward/record.url?scp=85140148477&partnerID=8YFLogxK
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U2 - 10.1007/978-3-031-12390-0_13
DO - 10.1007/978-3-031-12390-0_13
M3 - Chapter
C2 - 36255682
AN - SCOPUS:85140148477
T3 - Advances in Neurobiology
SP - 391
EP - 418
BT - Advances in Neurobiology
PB - Springer
ER -