Carnosine Excretion in Juvenile Amaurotic Idiocy

Levenson, Jeremy E.; Lindahl-Kiessling, Kerstin; S, Rayner

doi:10.1016/s0140-6736(64)92581-4

Cited by 16 publications

(4 citation statements)

References 6 publications

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“…An increased urinary excretion of carnosine has been reported by Bessman & Baldwin (3) and by Levenson et al (7) in children suffering from cerebro-macular degeneration. Perry et al (8) described two patients suffering from a progressive neurological disease who also showed carnosinuria.…”

mentioning

confidence: 70%

“…Up till now several cases of increased carnosine excretion have been described (3,7,8). In these reports the value given by Westall (16), determined for only 1 adult male, had been considered to be a normal carnosine excretion.…”

Section: Resultsmentioning

confidence: 99%

“…In a following article Tozci & Bessman (15) reported that the patients had an abnormal catabolism of histidine which resulted in increased synthesis and excretion of carnosine and anserine. Levenson et al (7) investigated 15 Swedish patients with this disease and found that at least some patients had occasionally increased excretion of carnosine. Perry et al (8) recently described two cases of carnosinuria.…”

Section: Discussionmentioning

confidence: 99%

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A Patient With a Deficiency of Serum‐carnosinase Activity

et al. 1969

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Summary A patient with severe mental retardation, generalized convulsions and electro‐encephalo‐graphic abnormalities has been described. A marked carnosinuria was observed on a regular diet as well as on a diet which was essentially free of carnosine. No carnosine was found in plasma of this patient on any occasion. The serum‐carnosinase activity was extremely diminished compared to control subjects. Oral loading with L‐carnosine resulted in a much greater excretion of the administered L‐carnosine compared to a control subject. The consanguineous parents of the patient also excreted large amounts of the dipeptide and had decreased serum‐carnosinase activities. Infusion with freshly prepared donor plasma, containing active carnosinase, resulted in a temporary elevation of the enzymic activity in serum and a decrease of carnosine excretion in urine.

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confidence: 70%

Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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A Patient With a Deficiency of Serum‐carnosinase Activity

et al. 1969

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“…In fact the concentrations of carnosine in human tissues and biological fluids are regulated by the activity of two dipeptidases: CNDP1 [ 12 ] and the cytosolic (CNDP2 or CN2) carnosinase [ 13 ], both of them members of the M20 metalloprotease family [ 14 ]. As it has been shown for the first time by Perry and colleagues, patients with carnosinase deficiency, a condition also known as carnosinemia, present an excess of carnosine in the urine (carnosinuria) and develop a progressive neurologic disorder characterized by severe mental defects and intellectual disability [ 15 , 16 , 17 ].…”

Section: Introductionmentioning

confidence: 99%

The Therapeutic Potential of Carnosine/Anserine Supplementation against Cognitive Decline: A Systematic Review with Meta-Analysis

et al. 2021

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Carnosine is a natural occurring endogenous dipeptide that was proposed as an anti-aging agent more than 20 years ago. Carnosine can be found at low millimolar concentrations at brain level and different preclinical studies have demonstrated its antioxidant, anti-inflammatory, and anti-aggregation activity with neuroprotective effects in animal models of Alzheimer’s disease (AD). A selective deficit of carnosine has also been linked to cognitive decline in AD. Different clinical studies have been conducted to evaluate the impact of carnosine supplementation against cognitive decline in elderly and AD subjects. We conducted a systematic review with meta-analysis, in accordance with the PRISMA guidelines coupled to the PICOS approach, to investigate the therapeutic potential of carnosine against cognitive decline and depressive symptoms in elderly subjects. We found five studies matching the selection criteria. Carnosine/anserine was administered for 12 weeks at a dose of 1 g/day and improved global cognitive function, whereas no effects were detected on depressive symptoms. These data suggest a preliminary evidence of clinical efficacy of carnosine against cognitive decline both in elderly subjects and mild cognitive impairment (MCI) patients, although larger and long-term clinical studies are needed in MCI patients (with or without depression) to confirm the therapeutic potential of carnosine.

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Carnosine and Inflammation

Caruso,

Maugeri,

Privitera

et al. 2024

Encyclopedia of Life Sciences

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Inflammation represents a complex biological response of the body to dangerous stimuli such as pathogens and involves different types of cells including immune cells (e.g., astrocytes and microglia), blood vessels, and molecular mediators. The main function of inflammation is to eradicate the causes responsible for cell injury, remove dead cells and damaged tissues, and allow the beginning of tissue repair. Carnosine is a dipeptide composed by β‐alanine and l ‐histidine possessing multiple functions that include anti‐aggregant, antioxidant, and anti‐inflammatory activities. Carnosine has also been shown to accelerate the wound‐healing process. Since numerous diseases are characterized by systemic and/or central inflammation such as cardiovascular diseases, type 2 diabetes mellitus, and neurodegenerative disorders, this molecule could be of great interest. Key Concepts Carnosine is a naturally occurring molecule characterized by a multimodal mechanism of action. The inflammatory process can be either short‐lived (acute; hours or days) or long‐lasting (chronic; months or years). Numerous systemic and neurodegenerative diseases show common pathogenic mechanisms including oxidative stress, inflammation and aggregation phenomena such as diabetes and Alzheimer disease. The versatile pharmacodynamic profile of carnosine including its anti‐inflammatory potential suggests a promising therapeutic potential in different diseases. Inflammation represents a complex biological response of the body to dangerous stimuli and involves different cell types such as immune cells (eg., astrocytes and microglia), blood vessels, and molecular mediators.

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Carnosine Excretion in Juvenile Amaurotic Idiocy

Cited by 16 publications

References 6 publications

A Patient With a Deficiency of Serum‐carnosinase Activity

A Patient With a Deficiency of Serum‐carnosinase Activity

The Therapeutic Potential of Carnosine/Anserine Supplementation against Cognitive Decline: A Systematic Review with Meta-Analysis

Carnosine and Inflammation

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