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Items: 13


Induced pluripotent stem cell models of Zellweger spectrum disorder show impaired peroxisome assembly and cell type-specific lipid abnormalities.

Wang XM, Yik WY, Zhang P, Lu W, Huang N, Kim BR, Shibata D, Zitting M, Chow RH, Moser AB, Steinberg SJ, Hacia JG.

Stem Cell Res Ther. 2015 Aug 29;6:158. doi: 10.1186/s13287-015-0149-3.


Association of common variations on chromosome 4q25 and left atrial volume in patients with atrial fibrillation.

Mints Y, Yarmohammadi H, Khurram IM, Hoyt H, Hansford R, Zimmerman SL, Steinberg SJ, Judge DP, Tomaselli GF, Calkins H, Zipunnikov V, Nazarian S.

Clin Med Insights Cardiol. 2015 May 13;9:39-45. doi: 10.4137/CMC.S21712. eCollection 2015.


The Pex1-G844D mouse: a model for mild human Zellweger spectrum disorder.

Hiebler S, Masuda T, Hacia JG, Moser AB, Faust PL, Liu A, Chowdhury N, Huang N, Lauer A, Bennett J, Watkins PA, Zack DJ, Braverman NE, Raymond GV, Steinberg SJ.

Mol Genet Metab. 2014 Apr;111(4):522-32. doi: 10.1016/j.ymgme.2014.01.008. Epub 2014 Jan 23.


The gene expression profiles of induced pluripotent stem cells from individuals with childhood cerebral adrenoleukodystrophy are consistent with proposed mechanisms of pathogenesis.

Wang XM, Yik WY, Zhang P, Lu W, Dranchak PK, Shibata D, Steinberg SJ, Hacia JG.

Stem Cell Res Ther. 2012 Oct 4;3(5):39. doi: 10.1186/scrt130.


Human and great ape red blood cells differ in plasmalogen levels and composition.

Moser AB, Steinberg SJ, Watkins PA, Moser HW, Ramaswamy K, Siegmund KD, Lee DR, Ely JJ, Ryder OA, Hacia JG.

Lipids Health Dis. 2011 Jun 17;10:101. doi: 10.1186/1476-511X-10-101.


Nonsense suppressor therapies rescue peroxisome lipid metabolism and assembly in cells from patients with specific PEX gene mutations.

Dranchak PK, Di Pietro E, Snowden A, Oesch N, Braverman NE, Steinberg SJ, Hacia JG.

J Cell Biochem. 2011 May;112(5):1250-8. doi: 10.1002/jcb.22979.


Identification of differences in human and great ape phytanic acid metabolism that could influence gene expression profiles and physiological functions.

Watkins PA, Moser AB, Toomer CB, Steinberg SJ, Moser HW, Karaman MW, Ramaswamy K, Siegmund KD, Lee DR, Ely JJ, Ryder OA, Hacia JG.

BMC Physiol. 2010 Oct 8;10:19. doi: 10.1186/1472-6793-10-19.


Identification of novel mutations and sequence variation in the Zellweger syndrome spectrum of peroxisome biogenesis disorders.

Yik WY, Steinberg SJ, Moser AB, Moser HW, Hacia JG.

Hum Mutat. 2009 Mar;30(3):E467-80. doi: 10.1002/humu.20932.


Bap31 enhances the endoplasmic reticulum export and quality control of human class I MHC molecules.

Ladasky JJ, Boyle S, Seth M, Li H, Pentcheva T, Abe F, Steinberg SJ, Edidin M.

J Immunol. 2006 Nov 1;177(9):6172-81.


Contiguous deletion of the X-linked adrenoleukodystrophy gene (ABCD1) and DXS1357E: a novel neonatal phenotype similar to peroxisomal biogenesis disorders.

Corzo D, Gibson W, Johnson K, Mitchell G, LePage G, Cox GF, Casey R, Zeiss C, Tyson H, Cutting GR, Raymond GV, Smith KD, Watkins PA, Moser AB, Moser HW, Steinberg SJ.

Am J Hum Genet. 2002 Jun;70(6):1520-31. Epub 2002 Apr 29.


X-linked adrenoleukodystrophy with non-diagnostic plasma very long chain fatty acids.

Kennedy CR, Allen JT, Fensom AH, Steinberg SJ, Wilson R.

J Neurol Neurosurg Psychiatry. 1994 Jun;57(6):759-61.

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