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Protein Name: Platelet-activating factor acetylhydrolase IB subunit alpha

UniprotKB/SwissProt ID: LIS1_HUMAN (P43034)

Gene Name: PAFAH1B1

Synonyms: LIS1, MDCR, MDS, PAFAHA

Organism: Homo sapiens (Human).

Function: Required for proper activation of Rho GTPases and actin polymerization at the leading edge of locomoting cerebellar neurons and postmigratory hippocampal neurons in response to calcium influx triggered via NMDA receptors. Non-catalytic subunit of an acetylhydrolase complex which inactivates platelet- activating factor (PAF) by removing the acetyl group at the SN-2 position (By similarity). Positively regulates the activity of the minus-end directed microtubule motor protein dynein. May enhance dynein-mediated microtubule sliding by targeting dynein to the microtubule plus end. Required for several dynein- and microtubule-dependent processes such as the maintenance of Golgi integrity, the peripheral transport of microtubule fragments and the coupling of the nucleus and centrosome. Required during brain development for the proliferation of neuronal precursors and the migration of newly formed neurons from the ventricular/subventricular zone toward the cortical plate. Neuronal migration involves a process called nucleokinesis, whereby migrating cells extend an anterior process into which the nucleus subsequently translocates. During nucleokinesis dynein at the nuclear surface may translocate the nucleus towards the centrosome by exerting force on centrosomal microtubules. May also play a role in other forms of cell locomotion including the migration of fibroblasts during wound healing.

Other Modifications: View all modification sites in dbPTM

Protein Subcellular Localization: Cytoplasm, cytoskeleton. Cytoplasm, cytoskeleton, microtubule organizing center, centrosome. Cytoplasm, cytoskeleton, spindle (By similarity). Nucleus membrane (Potential). Note=Redistributes to axons during neuronal development. Also localizes to the mi

Graphical Visualization of Ubiquitination Sites:
Overview of Protein Ubiquitination Sites with Functional and Structural Information
InterPro ID Domain
IPR001680
IPR006594
IPR011046
IPR013720
IPR015943


The ubiquitination sites of LIS1_HUMAN

No. Position Ubiquitinated Peptide Secondary Structure Solvent Accessibility Substrate Motifs PubMed ID
133AYSVFK K EAELDV HHHHHH H HHHCCC 51.17%21906983
276SKLNEA K EEFTSG EECCCC C CEEEEE 52.15%21906983
388GGPLGQ K RDPKEW EECCCC C CCCEEE 57.90%21906983
4147DFERTL K GHTDSV CEEEEE E CCCCCE 48.29%2190698

The interacting network mediated by proteins: LIS1_HUMAN


Disease:
Disease database Database Entry Disease information
KEGGH00268 Lissencephaly (LIS); Miller-Dieker syndrome (MDLS)
OMIM601545PLATELET-ACTIVATING FACTOR ACETYLHYDROLASE, ISOFORM 1B, ALPHA SUBUNIT; PAFAH1B1 ;;LIS1 GENE;
OMIM607432LISSENCEPHALY 1; LIS1 ;;LISSENCEPHALY SEQUENCE, ISOLATED; ILS;; LISSENCEPHALY, CLASSIC SUBCO
HPRD03329Lissencephaly sequence, isolated
HPRD03329Subcortical laminar heterotopia
Metabolic pathway:
Kegg map ID Pathway
map03008Ribosome biogenesis in eukaryotes