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Farber Lipogranulomatosis

Known as: Farber's Disease, Deficiency, N-Laurylsphingosine Deacylase, Deficiency, Acid Ceramidase 
A very rare autosomal recessive metabolic disorder affecting lipid metabolism. It is caused by mutations in the ASAH1 gene and is characterized by… Expand
National Institutes of Health

Papers overview

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Highly Cited
2012
Highly Cited
2012
Individuals sort into and out of economic environments based on their preferences and in response to relative prices. We… Expand
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Highly Cited
2007
Highly Cited
2007
This paper examines the construction equipment resale market to assess whether equipment produced by the world's largest… Expand
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Highly Cited
2007
Highly Cited
2007
BackgroundFarber Disease is an autosomal-recessively inherited, lysosomal storage disorder caused by acid ceramidase deficiency… Expand
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Highly Cited
2005
Highly Cited
2005
Using retroactive disclosures required by Statement of Financial Accounting Standards (SFAS) No. 131, we examine managers… Expand
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Highly Cited
2002
Highly Cited
2002
Ceramide is an important cellular lipid involved in signal transduction and the biosynthesis of complex sphingolipids. It can be… Expand
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Highly Cited
2001
Highly Cited
2001
Extinctions stand out amid the global catalog of environmental insults. Extinctions are irreversible and their rate is high and… Expand
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Highly Cited
2000
Highly Cited
2000
We have recently purified a rat brain membrane-bound nonlysosomal ceramidase (El Bawab, S., Bielawska, A., and Y. A. Hannun (1999… Expand
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Highly Cited
1995
Highly Cited
1995
Acid ceramidase (N-acylsphingosine deacylase, EC 3.5.1.23) is the lysosomal enzyme catalyzing the hydrolysis of ceramide to… Expand
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Highly Cited
1991
Highly Cited
1991
This paper studies career concerns -- concerns about the effects of current performance on future compensation -- and describes… Expand
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Highly Cited
1972
Highly Cited
1972
Ceramidase activity could not be demonstrated in the kidney and cerebellum from a deceased patient with Farber's disease, whereas… Expand
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