| Title |
4,5‐Dimethyl‐3‐hydroxy‐2[5H]‐furanone (sotolone) — The odour of maple syrup urine disease
|
|---|---|
| Published in |
Journal of Inherited Metabolic Disease, April 1999
|
| DOI | 10.1023/a:1005433516026 |
| Pubmed ID | |
| Authors |
F. Podebrad, M. Heil, S. Reichert, A. Mosandl, A. C. Sewell, H. Böhles |
| Abstract |
Maple syrup urine disease is an autosomal recessive inherited disorder of branched-chain amino acid metabolism due to deficiency of the branched-chain alpha-keto acid dehydrogenase complex. The disease was originally named after the characteristic sweet aroma, reminiscent of maple syrup, present in the body fluids of affected patients. Until now, the substance responsible for the odour has not been positively identified. Using enantioselective multidimensional gas chromatography-mass spectrometry (enantio-MDGC-MS), we could demonstrate that 4,5-dimethyl-3-hydroxy-2[5H]-furanone (sotolone), a well-known flavour impact compound present in fenugreek and lovage, was present in urine from seven patients with maple syrup urine disease. Urine samples from healthy control persons lacked sotolone. We have shown that sotolone is responsible for the characteristic odour of maple syrup urine disease and, since maple syrup also contains sotolone, the naming of this disease appears to be correct. |
X Demographics
The data shown below were collected from the profiles of 2 X users who shared this research output. Click here to find out more about how the information was compiled.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| United Kingdom | 1 | 50% |
| Unknown | 1 | 50% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Members of the public | 2 | 100% |
Mendeley readers
The data shown below were compiled from readership statistics for 66 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Czechia | 1 | 2% |
| Unknown | 65 | 98% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Student > Ph. D. Student | 11 | 17% |
| Student > Bachelor | 9 | 14% |
| Researcher | 9 | 14% |
| Student > Master | 8 | 12% |
| Other | 6 | 9% |
| Other | 8 | 12% |
| Unknown | 15 | 23% |
| Readers by discipline | Count | As % |
|---|---|---|
| Agricultural and Biological Sciences | 13 | 20% |
| Chemistry | 10 | 15% |
| Biochemistry, Genetics and Molecular Biology | 9 | 14% |
| Medicine and Dentistry | 9 | 14% |
| Engineering | 3 | 5% |
| Other | 7 | 11% |
| Unknown | 15 | 23% |
Attention Score in Context
This research output has an Altmetric Attention Score of 5. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 23 April 2024.
All research outputs
#6,729,311
of 24,451,065 outputs
Outputs from Journal of Inherited Metabolic Disease
#555
of 1,956 outputs
Outputs of similar age
#10,774
of 37,115 outputs
Outputs of similar age from Journal of Inherited Metabolic Disease
#2
of 9 outputs
Altmetric has tracked 24,451,065 research outputs across all sources so far. This one has received more attention than most of these and is in the 72nd percentile.
So far Altmetric has tracked 1,956 research outputs from this source. They receive a mean Attention Score of 4.8. This one has gotten more attention than average, scoring higher than 70% of its peers.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 37,115 tracked outputs that were published within six weeks on either side of this one in any source. This one has gotten more attention than average, scoring higher than 70% of its contemporaries.
We're also able to compare this research output to 9 others from the same source and published within six weeks on either side of this one. This one has scored higher than 7 of them.