Valerie Street Ph.D.
Research Associate Professor

Valerie's research interests employ genetics to understand the cause of inherited diseases. Currently, Valerie is focusing on the genetics of hearing loss in humans.

Valerie is also working with four large families affected by hearing loss. To facilitate this study, she has formed the Hereditary Equilibrium and Auditory Research (HEAR) Group.

In the past, the first research area focuses on understanding the genetic basis of the demyelinating neuropathy know as Charcot-Marie-Tooth disease (CMT). In Collaboration with Drs. Thomas Bird and Phillip Chance at the University of Washington, Valerie identified a new gene named LITAF/SIMPLE that underlies CMT type 1C. This gene may regulate protein degradation in the nervous system. Studies are underway to determine the function of this gene product. Funding for these studies has been provided by the Muscular Dsytrophy Association and the Charcot-Marie-Tooth Association.


Valerie Street's Publications

CMT Publications

Street VA, Bennett CL, Goldy JD, Shirk AJ, Kleopa KA, Tempel BL, Lipe HP, Scherer SS, Bird TD, Chance PF.
Mutation of a putative protein degradation gene LITAF/SIMPLE in Charcot-Marie-Tooth disease 1C.
Neurology. 2003 Jan 14;60(1):22-6.

Street VA, Meekins G, Lipe HP, Seltzer WK, Carter GT, Kraft GH, Bird TD.
Charcot-Marie-Tooth neuropathy: clinical phenotypes of four novel mutations in the MPZ and Cx 32 genes.
Neuromuscul Disord. 2002 Oct;12(7-8):643-50.

Street VA, Goldy JD, Golden AS, Tempel BL, Bird TD, Chance PF.
Mapping of Charcot-Marie-Tooth disease type 1C to chromosome 16p identifies a novel locus for demyelinating neuropathies.
Am J Hum Genet. 2002 Jan;70(1):244-50.

Other Publications

Street VA, McKee-Johnson JW, Fonseca RC, Tempel BL, Noben-Trauth K.
Mutations in a plasma membrane Ca2+-ATPase gene cause deafness in deafwaddler mice.
Nat Genet. 1998 Aug;19(4):390-4.

McKee-Johnson JW, Street VA, Erford SK, Robinson LC, Tempel BL.
Physical and genetic maps of the deafwaddler region on distal mouse Chr 6.
Genomics. 1998 May 1;49(3):371-7.

Street VA, Hopkins WF, Tempel BL.
Genomic structure, sequence, and physiological expression of mKv 1.5, a mouse potassium channel gene.
Epilepsy Res Suppl. 1996;12:165-75. No abstract available.

Street VA, Tempel BL.
Physical mapping of potassium channel gene clusters on mouse chromosomes three and six.
Genomics. 1997 Aug 15;44(1):110-7.

Street VA, Bosma MM, Demas VP, Regan MR, Lin DD, Robinson LC, Agnew WS, Tempel BL.
The type 1 inositol 1,4,5-trisphosphate receptor gene is altered in the opisthotonos mouse.
J Neurosci. 1997 Jan 15;17(2):635-45.

Street VA, Robinson LC, Erford SK, Tempel BL.
Molecular genetic analysis of distal mouse chromosome 6 defines gene order and positions of the deafwaddler and opisthotonos mutations.
Genomics. 1995 Sep 1;29(1):123-30.

Lock LF, Gilbert DJ, Street VA, Migeon MB, Jenkins NA, Copeland NG, Tempel BL.
Voltage-gated potassium channel genes are clustered in paralogous regions of the mouse genome.
Genomics. 1994 Apr;20(3):354-62.

Morton ME, Street VA, Nathanson NM.
Selective regulation of Gi alpha 1 expression and function in PC12 cells by cAMP.
J Neurosci. 1992 May;12(5):1839-46.

Migeon MB, Street VA, Demas VP, Tempel BL.
Cloning, sequence and chromosomal localization of MK6, a murine potassium channel gene.
Epilepsy Res Suppl. 1992;9:173-80; discussion 180-1. No abstract available.