Relative efficacy of nicotinamide treatment of a mouse model of infantile Niemann-Pick C1 disease

Marshall, Craig A.; Borbon, Ivan A.; Erickson, Robert P.

doi:10.1007/s13353-016-0367-0

Cited by 6 publications

(2 citation statements)

References 24 publications

(26 reference statements)

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“…This result points out how the use of this vitamin in the diet could be useful for the treatment of NPC patients. Furthermore, beneficial effect of nicotinamide is also reported in counteracting cognitive impairment and enhancing survival [ 127 ].…”

Section: Additional Non-pharmacologic Approaches For Neurodegenerationmentioning

confidence: 99%

Neurodegeneration in Niemann–Pick Type C Disease: An Updated Review on Pharmacological and Non-Pharmacological Approaches to Counteract Brain and Cognitive Impairment

Cariati

Masuelli

Bei

et al. 2021

IJMS

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Niemann–Pick type C (NPC) disease is an autosomal recessive storage disorder, characterized by abnormal sequestration of unesterified cholesterol in the late endo-lysosomal system of cells. Progressive neurological deterioration and the onset of symptoms, such as ataxia, seizures, cognitive decline, and severe dementia, are pathognomonic features of the disease. In addition, different pathological similarities, including degeneration of hippocampal and cortical neurons, hyperphosphorylated tau, and neurofibrillary tangle formation, have been identified between NPC disease and other neurodegenerative pathologies. However, the underlying pathophysiological mechanisms are not yet well understood, and even a real cure to counteract neurodegeneration has not been identified. Therefore, the combination of current pharmacological therapies, represented by miglustat and cyclodextrin, and non-pharmacological approaches, such as physical exercise and appropriate diet, could represent a strategy to improve the quality of life of NPC patients. Based on this evidence, in our review we focused on the neurodegenerative aspects of NPC disease, summarizing the current knowledge on the molecular and biochemical mechanisms responsible for cognitive impairment, and suggesting physical exercise and nutritional treatments as additional non-pharmacologic approaches to reduce the progression and neurodegenerative course of NPC disease.

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Section: Additional Non-pharmacologic Approaches For Neurodegenerationmentioning

confidence: 99%

Neurodegeneration in Niemann–Pick Type C Disease: An Updated Review on Pharmacological and Non-Pharmacological Approaches to Counteract Brain and Cognitive Impairment

Cariati

Masuelli

Bei

et al. 2021

IJMS

View full text Add to dashboard Cite

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“…AMPK increases the activity of NAMPT (51). This process catalyzes the conversion of nicotinamide to nicotinamide mononucleotide (69), increases NAD + levels (69), decreases levels of nicotinamide (70), an inhibitor of SIRT1 (69, 71–73), and leads to SIRT1 transcription (13, 48, 74). As a result of increasing the intracellular NAD + /NADH ratio, AMPK leads to deacetylation of the SIRT1 targets peroxisome proliferator-activated receptor-gamma coactivator 1 (PGC-1α) and forkhead transcription factors that include FoxO1 (37) and FoxO3a (75).…”

Section: Sirt1 As a Target For Vascular Diseasementioning

confidence: 99%

Harnessing the Power of SIRT1 and Non-coding RNAs in Vascular Disease

Maiese¹

2017

CNR

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Noncommunicable diseases (NCDs) contribute to a significant amount of disability and death in the world. Of these disorders, vascular disease is ranked high, falls within the five leading causes of death, and impacts multiple other disease entities such as those of the cardiac system, nervous system, and metabolic disease. Targeting the silent mating type information regulation 2 homolog 1 (Saccharomyces cerevisiae) (SIRT1) pathway and the modulation of micro ribonucleic acids (miRNAs) may hold great promise for the development of novel strategies for the treatment of vascular disease since each of these pathways are highly relevant to cardiac and nervous system disorders as well as to metabolic dysfunction. SIRT1 is vital in determining the course of stem cell development and the survival, metabolism, and life span of differentiated cells that are overseen by both autophagy and apoptosis. SIRT1 interfaces with a number of pathways that involve forkhead transcription factors, mechanistic of rapamycin (mTOR), AMP activated protein kinase (AMPK) and Wnt1 inducible signaling pathway protein 1 (WISP1) such that the level of activity of SIRT1 can become a critical determinant for biological and clinical outcomes. The essential fine control of SIRT1 is directly tied to the world of non-coding RNAs that ultimately oversee SIRT1 activity to either extend or end cellular survival. Future studies that can further elucidate the crosstalk between SIRT1 and non-coding RNAs should serve well our ability to harness the power of SIRT1 and non-coding RNAs for the treatment of vascular disorders.

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Sirtuins, mitochondria, and exercise in health and disease

Dabke

2021

Sirtuin Biology in Medicine

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Relative efficacy of nicotinamide treatment of a mouse model of infantile Niemann-Pick C1 disease

Cited by 6 publications

References 24 publications

Neurodegeneration in Niemann–Pick Type C Disease: An Updated Review on Pharmacological and Non-Pharmacological Approaches to Counteract Brain and Cognitive Impairment

Neurodegeneration in Niemann–Pick Type C Disease: An Updated Review on Pharmacological and Non-Pharmacological Approaches to Counteract Brain and Cognitive Impairment

Harnessing the Power of SIRT1 and Non-coding RNAs in Vascular Disease

Sirtuins, mitochondria, and exercise in health and disease

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