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Items: 21

1.

Morpholino Antisense Oligomers as a Potential Therapeutic Option for the Correction of Alternative Splicing in PMD, SPG2, and HEMS.

Tantzer S, Sperle K, Kenaley K, Taube J, Hobson GM.

Mol Ther Nucleic Acids. 2018 Sep 7;12:420-432. doi: 10.1016/j.omtn.2018.05.019. Epub 2018 Jul 5.

2.

Altered PLP1 splicing causes hypomyelination of early myelinating structures.

Kevelam SH, Taube JR, van Spaendonk RM, Bertini E, Sperle K, Tarnopolsky M, Tonduti D, Valente EM, Travaglini L, Sistermans EA, Bernard G, Catsman-Berrevoets CE, van Karnebeek CD, Ƙstergaard JR, Friederich RL, Fawzi Elsaid M, Schieving JH, Tarailo-Graovac M, Orcesi S, Steenweg ME, van Berkel CG, Waisfisz Q, Abbink TE, van der Knaap MS, Hobson GM, Wolf NI.

Ann Clin Transl Neurol. 2015 Jun;2(6):648-61. doi: 10.1002/acn3.203. Epub 2015 May 1.

3.

Complex genomic rearrangements at the PLP1 locus include triplication and quadruplication.

Beck CR, Carvalho CM, Banser L, Gambin T, Stubbolo D, Yuan B, Sperle K, McCahan SM, Henneke M, Seeman P, Garbern JY, Hobson GM, Lupski JR.

PLoS Genet. 2015 Mar 6;11(3):e1005050. doi: 10.1371/journal.pgen.1005050. eCollection 2015 Mar.

4.

PMD patient mutations reveal a long-distance intronic interaction that regulates PLP1/DM20 alternative splicing.

Taube JR, Sperle K, Banser L, Seeman P, Cavan BC, Garbern JY, Hobson GM.

Hum Mol Genet. 2014 Oct 15;23(20):5464-78. doi: 10.1093/hmg/ddu271. Epub 2014 Jun 1.

5.

Gait abnormalities and progressive myelin degeneration in a new murine model of Pelizaeus-Merzbacher disease with tandem genomic duplication.

Clark K, Sakowski L, Sperle K, Banser L, Landel CP, Bessert DA, Skoff RP, Hobson GM.

J Neurosci. 2013 Jul 17;33(29):11788-99. doi: 10.1523/JNEUROSCI.1336-13.2013.

6.

The role of calcium in the activation of estrogen receptor-alpha.

Divekar SD, Storchan GB, Sperle K, Veselik DJ, Johnson E, Dakshanamurthy S, Lajiminmuhip YN, Nakles RE, Huang L, Martin MB.

Cancer Res. 2011 Mar 1;71(5):1658-68. doi: 10.1158/0008-5472.CAN-10-1899. Epub 2011 Jan 6.

7.

Deletion of a splicing enhancer disrupts PLP1/DM20 ratio and myelin stability.

Wang E, Dimova N, Sperle K, Huang Z, Lock L, McCulloch MC, Edgar JM, Hobson GM, Cambi F.

Exp Neurol. 2008 Dec;214(2):322-30. doi: 10.1016/j.expneurol.2008.09.001. Epub 2008 Sep 18.

PMID:
18835559
8.

Heterogeneous duplications in patients with Pelizaeus-Merzbacher disease suggest a mechanism of coupled homologous and nonhomologous recombination.

Woodward KJ, Cundall M, Sperle K, Sistermans EA, Ross M, Howell G, Gribble SM, Burford DC, Carter NP, Hobson DL, Garbern JY, Kamholz J, Heng H, Hodes ME, Malcolm S, Hobson GM.

Am J Hum Genet. 2005 Dec;77(6):966-87. Epub 2005 Oct 19.

9.

Spastic paraplegia type 2 associated with axonal neuropathy and apparent PLP1 position effect.

Lee JA, Madrid RE, Sperle K, Ritterson CM, Hobson GM, Garbern J, Lupski JR, Inoue K.

Ann Neurol. 2006 Feb;59(2):398-403.

PMID:
16374829
10.

PLP1 alternative splicing in differentiating oligodendrocytes: characterization of an exonic splicing enhancer.

Wang E, Huang Z, Hobson GM, Dimova N, Sperle K, McCullough A, Cambi F.

J Cell Biochem. 2006 Apr 1;97(5):999-1016.

PMID:
16288477
11.

Splice-site contribution in alternative splicing of PLP1 and DM20: molecular studies in oligodendrocytes.

Hobson GM, Huang Z, Sperle K, Sistermans E, Rogan PK, Garbern JY, Kolodny E, Naidu S, Cambi F.

Hum Mutat. 2006 Jan;27(1):69-77.

PMID:
16287154
12.

Schwann cell expression of PLP1 but not DM20 is necessary to prevent neuropathy.

Shy ME, Hobson G, Jain M, Boespflug-Tanguy O, Garbern J, Sperle K, Li W, Gow A, Rodriguez D, Bertini E, Mancias P, Krajewski K, Lewis R, Kamholz J.

Ann Neurol. 2003 Mar;53(3):354-65.

13.

A PLP splicing abnormality is associated with an unusual presentation of PMD.

Hobson GM, Huang Z, Sperle K, Stabley DL, Marks HG, Cambi F.

Ann Neurol. 2002 Oct;52(4):477-88.

PMID:
12325077
14.

Molecular identification and analysis of a novel human corticotropin-releasing factor (CRF) receptor: the CRF2gamma receptor.

Kostich WA, Chen A, Sperle K, Largent BL.

Mol Endocrinol. 1998 Aug;12(8):1077-85.

PMID:
9717834
15.

Rapid generation of stable cell lines expressing corticotropin-releasing hormone receptor for drug discovery.

Horlick RA, Sperle K, Breth LA, Reid CC, Shen ES, Robbins AK, Cooke GM, Largent BL.

Protein Expr Purif. 1997 Apr;9(3):301-8.

PMID:
9126600
16.
18.
19.

Recombination in mouse L cells between DNA introduced into cells and homologous chromosomal sequences.

Lin FL, Sperle K, Sternberg N.

Proc Natl Acad Sci U S A. 1985 Mar;82(5):1391-5.

21.

Homologous recombination in mouse L cells.

Lin FL, Sperle K, Sternberg N.

Cold Spring Harb Symp Quant Biol. 1984;49:139-49. No abstract available.

PMID:
6597752

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