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FLASH GENE

Symbol

FEZF2

contributors: mct - updated : 10-09-2015

HGNC name	FEZ family zinc finger 2
HGNC id	13506

FLASH GENE DNA RNA EXPRESSION PROTEIN DISORDER ANIMAL & CELL MODELS

RNA

TRANSCRIPTS	type	messenger

identification nb exons type bp product Protein kDa AA specific expression Year Pubmed NM_018008 5 - 2095 48 459 - 2011 21471212

EXPRESSION

Type	restricted

constitutive of

expressed in	(based on citations)
organ(s)	System Organ level 1 Organ level 2 Organ level 3 Organ level 4 Level Pubmed Species Stage Rna symbol Nervous brain forebrain cerebral cortex     Visual eye

cells

System Cell Pubmed Species Stage Rna symbol Nervous pyramidal cell

cell lineage

cell lines

fluid/secretion

at STAGE

physiological period

embryo

Text	early during embryonic development, ventricular zone progenitor cells

PROTEIN

PHYSICAL PROPERTIES

STRUCTURE

motifs/domains	N-terminal domain with homology to the canonical engrailed repressor motif
	a KRAB domain
	C-terminal DNA binding domain containing six C2H2 zinc-finger repeats

HOMOLOGY

Homologene

FAMILY

krueppel C2H2-type zinc-finger protein family

CATEGORY

regulatory

SUBCELLULAR LOCALIZATION	intracellular
	intracellular,cytoplasm,organelle,mitochondria
	intracellular,nucleus

basic FUNCTION	transcriptional repressor, required for the differentiation of projection neurons in cortical layer 5
	is essential for specification of the subcerebral projection neurons in the neocortex
	FEZF1 and FEZF2 control likely the rostro-caudal patterning of the diencephalon
	FEZF1 and FEZF2 bind to and directly repress the promoter activity of HES5
	FEZF1 and FEZF2 control differentiation of neural stem cells by repressing HES5 and, in turn, by derepressing neurogenin 2 in the forebrain
	playing a critical role in layer- and neuronal subtype-specific patterning of cortical axonal projections and dendritic morphologies
	required for the birth and specification of corticospinal motor neurons
	directs the differentiation of corticofugal neurons from striatal progenitors
	conserved gene critical for vertebrate forebrain development
	FEZF1 and FEZF2, regulate the identity of main olfactory epithelium (MOE) sensory neurons and are essential for the survival of vomeronasal organ (VNO), neurons respectively
	is important for early development of the posterior hypothalamus
	is required for the development of excitatory neurons and nuclear morphology in the lateral and basolateral nuclei
	FEZF1 and FEZF2 as important regulators of olfactory system development and sensory neuron identity
	controlling neurogenesis and cell fate in the developing mammalian nervous system

CELLULAR PROCESS

nucleotide, transcription

PHYSIOLOGICAL PROCESS

PATHWAY

metabolism

signaling

a component

INTERACTION

DNA

RNA

small molecule

protein	BCL11B is a major downstream effector of FEZF2 in regulating the extension of axons toward subcortical targets and can rescue the axonal phenotype of FEZF2 mutants
	TBR1 is a direct transcriptional repressor of FEZF2 and a negative regulator of corticospinal tract formation that restricts the laminar origin of corticospinal axons specifically to L5
	axon guidance receptor EPHB1 is a target of FEZF2 necessary to execute the ipsilateral extension of the corticospinal tract

cell & other

REGULATION

Other

regulated by SOX5 and TBR1 (both SOX5 and TBR1 are required in the regulation of FEZF2 transcription)

ASSOCIATED DISORDERS

corresponding disease(s)

Susceptibility

Variant & Polymorphism

Candidate gene	candidate for evaluation in human microcephaly
Marker
Therapy target

ANIMAL & CELL MODELS

	Fezf2(-/-) neurons adopt the fate of callosal projection neurons as assessed by their axonal projections, electrophysiological properties, and acquisition of Satb2 expression