Ehud Goldin
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Explore the profile of Ehud Goldin including associated specialties, affiliations and a list of published articles.
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40
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1409
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Recent Articles
1.
Aflaki E, Stubblefield B, Maniwang E, Lopez G, Moaven N, Goldin E, et al.
Sci Transl Med
. 2014 Jun;
6(240):240ra73.
PMID: 24920659
Gaucher disease is caused by an inherited deficiency of glucocerebrosidase that manifests with storage of glycolipids in lysosomes, particularly in macrophages. Available cell lines modeling Gaucher disease do not demonstrate...
2.
Panicker L, Miller D, Park T, Patel B, Azevedo J, Awad O, et al.
Proc Natl Acad Sci U S A
. 2012 Oct;
109(44):18054-9.
PMID: 23071332
Gaucher disease (GD) is an autosomal recessive disorder caused by mutations in the acid β-glucocerebrosidase gene. To model GD, we generated human induced pluripotent stem cells (hiPSC), by reprogramming skin...
3.
Xiao J, Westbroek W, Motabar O, Lea W, Hu X, Velayati A, et al.
J Med Chem
. 2012 Jul;
55(17):7546-59.
PMID: 22834902
Pompe disease is an autosomal recessive lysosomal storage disorder (LSD) caused by deficiency of the lysosomal enzyme acid α-glucosidase (GAA). Many disease-causing mutated GAA retain enzymatic activity but are not...
4.
Harzer K, Blech-Hermoni Y, Goldin E, Felderhoff-Mueser U, Igney C, Sidransky E, et al.
Biochem Biophys Res Commun
. 2012 Jun;
423(2):308-12.
PMID: 22659419
Beta-glucosidase 1 (GBA1; lysosomal glucocerebrosidase) and β-glucosidase 2 (GBA2, non-lysosomal glucocerebrosidase) both have glucosylceramide as a main natural substrate. The enzyme-deficient conditions with glucosylceramide accumulation are Gaucher disease (GBA-/- in...
5.
Tamargo R, Velayati A, Goldin E, Sidransky E
Mol Genet Metab
. 2012 Jun;
106(3):257-63.
PMID: 22652185
Saposin C is one of four homologous proteins derived from sequential cleavage of the saposin precursor protein, prosaposin. It is an essential activator for glucocerebrosidase, the enzyme deficient in Gaucher...
6.
Patnaik S, Zheng W, Choi J, Motabar O, Southall N, Westbroek W, et al.
J Med Chem
. 2012 Jun;
55(12):5734-48.
PMID: 22646221
A major challenge in the field of Gaucher disease has been the development of new therapeutic strategies including molecular chaperones. All previously described chaperones of glucocerebrosidase are enzyme inhibitors, which...
7.
Marugan J, Huang W, Motabar O, Zheng W, Xiao J, Patnaik S, et al.
Medchemcomm
. 2012 May;
3(1):56-60.
PMID: 22606365
Small molecule chaperones are a promising therapeutic approach for the Lysosomal Storage Disorders (LSDs). Here, we report the discovery of a new series of non-iminosugar glucocerebrosidase inhibitors with chaperone capacity,...
8.
Goldin E, Zheng W, Motabar O, Southall N, Choi J, Marugan J, et al.
PLoS One
. 2012 Jan;
7(1):e29861.
PMID: 22272254
Gaucher disease (GD), the most common lysosomal storage disorder, results from the inherited deficiency of the lysosomal enzyme glucocerebrosidase (GCase). Previously, wildtype GCase was used for high throughput screening (HTS)...
9.
Motabar O, Goldin E, Leister W, Liu K, Southall N, Huang W, et al.
Anal Bioanal Chem
. 2011 Oct;
402(2):731-9.
PMID: 22033823
Glucocerebrosidase is a lysosomal enzyme that catalyzes the hydrolysis of glucosylceramide to form ceramide and glucose. A deficiency of lysosomal glucocerebrosidase due to genetic mutations results in Gaucher disease, in...
10.
Velayati A, DePaolo J, Gupta N, Choi J, Moaven N, Westbroek W, et al.
Hum Mutat
. 2011 Jul;
32(11):1232-8.
PMID: 21796727
Lysosomal integral membrane protein type 2 (LIMP-2) is responsible for proper sorting and lysosomal targeting of glucocerebrosidase, the enzyme deficient in Gaucher disease (GD). Mutations in the gene for LIMP-2,...