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PPFIBP1

Domain

The N-terminal coiled coil regions mediate homodimerization preferentially and heterodimerization type beta/beta. The C-terminal, non-coiled coil regions mediate heterodimerization type beta/alpha and interaction with S100A4.

Function

May regulate the disassembly of focal adhesions. Did not bind receptor-like tyrosine phosphatases type 2A.

Involvement in disease

Neurodevelopmental disorder with seizures, microcephaly, and brain abnormalities

NEDSMBA

An autosomal recessive disorder characterized by global developmental delay, severe to profound intellectual disability, progressive microcephaly, refractory early-onset epilepsy, white matter abnormalities, and periventricular calcifications.

None

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

Sequence Similarities

Belongs to the liprin family. Liprin-beta subfamily.

Tissue Specificity

Widely expressed. Absent in liver.

Cellular localization

Alternative names

KIAA1230, PPFIBP1, Liprin-beta-1, Protein tyrosine phosphatase receptor type f polypeptide-interacting protein-binding protein 1, hSGT2, PTPRF-interacting protein-binding protein 1

swissprot:Q86W92 omim:603141 entrezGene:8496