JavaScript is disabled in your browser. Please enable JavaScript to view this website.

AP4E1

Function

Component of the adaptor protein complex 4 (AP-4). Adaptor protein complexes are vesicle coat components involved both in vesicle formation and cargo selection. They control the vesicular transport of proteins in different trafficking pathways (PubMed:10066790, PubMed:10436028). AP-4 forms a non clathrin-associated coat on vesicles departing the trans-Golgi network (TGN) and may be involved in the targeting of proteins from the trans-Golgi network (TGN) to the endosomal-lysosomal system. It is also involved in protein sorting to the basolateral membrane in epithelial cells and the proper asymmetric localization of somatodendritic proteins in neurons. AP-4 is involved in the recognition and binding of tyrosine-based sorting signals found in the cytoplasmic part of cargos, but may also recognize other types of sorting signal (Probable).

Involvement in disease

Spastic paraplegia 51, autosomal recessive

SPG51

A form of spastic paraplegia, a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. SPG51 is a non-progressive disorder of movement and/or posture resulting from defects in the developing central nervous system. Affected individuals manifest motor and posture impairments often associated with epilepsy and disturbances of cognition, behavior, sensation, and communication.

None

The disease is caused by variants affecting the gene represented in this entry.

Stuttering, familial persistent 1

STUT1

A familial form of stuttering, a disturbance in the normal fluency and time patterning of speech, characterized by frequent repetitions or prolongations of sounds or syllables, and by interruptions of speech known as blocks. STUT1 inheritance is autosomal dominant.

None

The disease is caused by variants affecting the gene represented in this entry.

Sequence Similarities

Belongs to the adaptor complexes large subunit family.

Tissue Specificity

Widely expressed.

Cellular localization

Alternative names

AP-4 complex subunit epsilon-1, AP-4 adaptor complex subunit epsilon, Adaptor-related protein complex 4 subunit epsilon-1, Epsilon subunit of AP-4, Epsilon-adaptin, AP4E1

swissprot:Q9UPM8 entrezGene:23431 omim:607244