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Rabbit Anti-ZNF259/ZPR1 antibody
ZPR1 is a zinc finger-containing protein that is capable of binding to the intracellular tyrosine kinase domain of the epidermal growth factor receptor. Stimulation of mammalian cells with epidermal growth factor reduces ZPR1 affinity for the EGFR and leads to an accumulation of the protein in the nucleus. The ZPR1 zinc finger is necessary for its association with the EGFR.
Function:
Acts as a signaling molecule that communicates proliferative growth signals from the cytoplasm to the nucleus. Plays a role for the localization and accumulation of the survival motor neuron protein SMN1 in sub-nuclear bodies, including gems and Cajal bodies. Induces neuron differentiation and stimulates axonal growth and formation of growth cone in spinal cord motor neurons. Plays a role in the splicing of cellular pre-mRNAs. May be involved in H2O(2)-induced neuronal cell death.
Subunit:
Component of an import snRNP complex composed of KPNB1, SNUPN, SMN1 and ZNF259. Interacts (via SLCterminal region) with SMN1 (via SLCterminal region); the interaction occurs after treatment with serum By similarity. Interacts with elongation factor 1-alpha EEF1A1; the interaction occurs in a epidermal growth factor (EGF)-dependent manner. Interacts (via zinc fingers) with EGFR (via SLCterminal cytoplasmic kinase domain); the interaction is negatively regulated in response to epidermal growth factor (EGF) stimulation and EGFR kinase activity. May also bind to the PDGFR receptor.
Subcellular Location:
Cytoplasm. Nucleus > nucleolus. Translocates to the nucleolus after treatment with mitogens.
Tissue Specificity:
Expressed in brain. Expressed in the spinal cord motor neurons (at protein level). Expressed in spleen, liver, muscle, kidney and testis. Expressed in the frontal cortex, cornus ammonis, dentate gyrus of the hippocampus and in Purkinje cells of the cerebellum.
DISEASE:
May contribute to the severity of spinal muscular atrophy by increasing spinal motor neurons degeneration (Ref.7).
Disruption phenotype Die during early embryonic development. Embryos show growth delay, failed to form normal trophectoderm and to expand the inner cell mass.
Similarity:
Belongs to the ZPR1 family.
SWISS:
O75312
Gene ID:
8882
Database links:
Entrez Gene: 8882 Human
Entrez Gene: 22687 Mouse
Entrez Gene: 500989 Rat
Omim: 603901 Human
SwissProt: O75312 Human
SwissProt: Q62384 Mouse
Unigene: 7165 Human
Unigene: 17519 Mouse
Unigene: 102011 Rat
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