The transcription factor Bhlhb4 is required for rod bipolar cell maturation.

Abstract:

:Retinal bipolar cells are essential to the transmission of light information. Although bipolar cell dysfunction can result in blindness, little is known about the factors required for bipolar cell development and functional maturation. The basic helix-loop-helix (bHLH) transcription factor Bhlhb4 was found to be expressed in rod bipolar cells (RB). Electroretinograms (ERGs) in the adult Bhlhb4 knockout (Bhlhb4(-/-)) showed that the loss of Bhlhb4 resulted in disrupted rod signaling and profound retinal dysfunction resembling human congenital stationary night blindness (CSNB), characterized by the loss of the scotopic ERG b-wave. A depletion of inner nuclear layer (INL) cells in the adult Bhlhb4 knockout has been ascribed to the abolishment of the RB cell population during postnatal development. Other retinal cell populations including photoreceptors were unaltered. The timing of RB cell depletion in the Bhlhb4(-/-) mouse suggests that Bhlhb4 is essential for RB cell maturation.

journal_name

Neuron

journal_title

Neuron

authors

Bramblett DE,Pennesi ME,Wu SM,Tsai MJ

doi

10.1016/j.neuron.2004.08.032

subject

Has Abstract

pub_date

2004-09-16 00:00:00

pages

779-93

issue

6

eissn

0896-6273

issn

1097-4199

pii

S0896627304005410

journal_volume

43

pub_type

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