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Protein Page:
PHOX2A (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitylation
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

PHOX2A May be involved in regulating the specificity of expression of the catecholamine biosynthetic genes. Acts as a transcription activator/factor. Could maintain the noradrenergic phenotype. Defects in PHOX2A are the cause of congenital fibrosis of extraocular muscles type 2 (CFEOM2). CFEOM encompasses several different inherited strabismus syndromes characterized by congenital restrictive ophthalmoplegia affecting extraocular muscles innervated by the oculomotor and/or trochlear nerves. CFEOM is characterized clinically by anchoring of the eyes in downward gaze, ptosis, and backward tilt of the head. CFEOM2 may result from the aberrant development of the oculomotor (nIII), trochlear (nIV) and abducens (nVI) cranial nerve nuclei. Belongs to the paired homeobox family. Note: This description may include information from UniProtKB.
Protein type: Transcription factor; DNA-binding
Chromosomal Location of Human Ortholog: 11q13.2
Cellular Component: nuclear chromatin
Molecular Function: transcription factor activity
Biological Process: locus ceruleus development; midbrain development; oculomotor nerve formation; positive regulation of transcription from RNA polymerase II promoter; regulation of respiratory gaseous exchange; somatic motor neuron differentiation; sympathetic nervous system development; transcription, DNA-dependent; trochlear nerve formation
Disease: Fibrosis Of Extraocular Muscles, Congenital, 2
Reference #:  O14813 (UniProtKB)
Alt. Names/Synonyms: aristaless homeobox homolog; Aristaless homeobox protein homolog; ARIX; arix homeodomain protein; ARIX1 homeodomain protein; CFEOM2; FEOM2; MGC52227; NCAM2; Paired mesoderm homeobox protein 2A; Paired-like homeobox 2A; PHOX2A; PHX2A; PMX2A
Gene Symbols: PHOX2A
Molecular weight: 29,653 Da
Basal Isoelectric point: 8.98  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

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Sites Implicated In
transcription, inhibited: S153‑p, S206‑p
activity, inhibited: S153‑p
molecular association, regulation: S153‑p, S206‑p
phosphorylation: S202‑p, S206‑p, S208‑p

Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend

Show Multiple Sequence Alignment


LTP: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


HTP: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


1 1 S202‑p PPAPGLAsPRLsPsP
1 1 S206‑p GLAsPRLsPsPLPVA
1 2 S208‑p AsPRLsPsPLPVALG

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