Gigaxonin is associated with the Golgi and dimerises via its BTB domain

TY - JOUR

T1 - Gigaxonin is associated with the Golgi and dimerises via its BTB domain

AU - Cullen, V C

AU - Brownlees, J

AU - Banner, S

AU - Anderton, B H

AU - Leigh, P N

AU - Shaw, C E

AU - Miller, C C J

PY - 2004/4/9

Y1 - 2004/4/9

N2 - Mutations in the gigaxonin gene cause giant axonal neuropathy. The amino-terminus of gigaxonin contains a BTB domain but no binding partners for this domain have so far been identified. Here, we demonstrate that the gigaxonin BTB domain forms homodimers. Other BTB-bearing proteins have also been shown to dimerise via their BTB domains with the dinners then capable of interacting with other ligands. Thus, the gigaxonin BTB domain may function in a similar manner. We also demonstrate that gigaxonin is expressed in a wide variety of neuronal cell types where a significant proportion exists in cell bodies. Confocal microscope studies of gigaxonin-transfected COS-7 cells and cultured neurones revealed that a proportion of gigaxonin localises to the Golgi and endoplasmic reticulum.

AB - Mutations in the gigaxonin gene cause giant axonal neuropathy. The amino-terminus of gigaxonin contains a BTB domain but no binding partners for this domain have so far been identified. Here, we demonstrate that the gigaxonin BTB domain forms homodimers. Other BTB-bearing proteins have also been shown to dimerise via their BTB domains with the dinners then capable of interacting with other ligands. Thus, the gigaxonin BTB domain may function in a similar manner. We also demonstrate that gigaxonin is expressed in a wide variety of neuronal cell types where a significant proportion exists in cell bodies. Confocal microscope studies of gigaxonin-transfected COS-7 cells and cultured neurones revealed that a proportion of gigaxonin localises to the Golgi and endoplasmic reticulum.

UR - http://www.scopus.com/inward/record.url?scp=1842556678&partnerID=8YFLogxK

U2 - 10.1097/00001756-200404090-00028

DO - 10.1097/00001756-200404090-00028

M3 - Article

VL - 15

SP - 873

EP - 876

JO - Neuroreport

JF - Neuroreport

IS - 5

ER -