| Title |
Disorders of glycoprotein degradation
|
|---|---|
| Published in |
Journal of Inherited Metabolic Disease, July 1990
|
| DOI | 10.1007/bf01799510 |
| Pubmed ID | |
| Authors |
M. Cantz, B. Ulrich‐Bott |
| Abstract |
The intracellular degradation of glycoproteins occurs predominantly in the lysosomes through the concerted action of proteases and glycosidases. Genetic defects in any of the enzymes cleaving the oligosaccharide side chains lead to specific diseases because of an excessive lysosomal accumulation of partially degraded material, mostly oligosaccharides. This paper presents an overview of the biochemistry and the clinical spectrum of this group of diseases including sialidosis, galactosialidosis, alpha- and beta-mannosidosis, fucosidosis, aspartylglucosaminuria, and alpha-N-acetylgalactosaminidase deficiency (Schindler disease). In addition, the sialic acid storage disorder (Salla disease) which is caused by a defect in the lysosomal transport of this acidic monosaccharide is included because of functional and clinical correlations. |
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 % |
|---|---|---|
| Unknown | 2 | 100% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Practitioners (doctors, other healthcare professionals) | 1 | 50% |
| Science communicators (journalists, bloggers, editors) | 1 | 50% |
Mendeley readers
The data shown below were compiled from readership statistics for 14 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 14 | 100% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Student > Bachelor | 5 | 36% |
| Researcher | 4 | 29% |
| Professor | 1 | 7% |
| Student > Master | 1 | 7% |
| Student > Ph. D. Student | 1 | 7% |
| Other | 0 | 0% |
| Unknown | 2 | 14% |
| Readers by discipline | Count | As % |
|---|---|---|
| Biochemistry, Genetics and Molecular Biology | 5 | 36% |
| Agricultural and Biological Sciences | 2 | 14% |
| Medicine and Dentistry | 2 | 14% |
| Chemistry | 2 | 14% |
| Neuroscience | 1 | 7% |
| Other | 0 | 0% |
| Unknown | 2 | 14% |
Attention Score in Context
This research output has an Altmetric Attention Score of 7. 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 16 April 2024.
All research outputs
#4,611,824
of 23,445,423 outputs
Outputs from Journal of Inherited Metabolic Disease
#273
of 1,880 outputs
Outputs of similar age
#1,799
of 15,774 outputs
Outputs of similar age from Journal of Inherited Metabolic Disease
#1
of 13 outputs
Altmetric has tracked 23,445,423 research outputs across all sources so far. Compared to these this one has done well and is in the 80th percentile: it's in the top 25% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 1,880 research outputs from this source. They receive a mean Attention Score of 4.7. This one has done well, scoring higher than 85% 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 15,774 tracked outputs that were published within six weeks on either side of this one in any source. This one has done well, scoring higher than 88% of its contemporaries.
We're also able to compare this research output to 13 others from the same source and published within six weeks on either side of this one. This one has done particularly well, scoring higher than 92% of its contemporaries.