Huntingtin Interacting Protein 1 Related Protein (HIP1R)

Huntingtin Interacting Protein 1 is a membrane-associated protein that interacts with huntingtin (HTT), the protein altered in Huntington disease (HD). The highest degree of similarity occurs in the C-terminal region, which shows considerable homology to the cytoskeletal protein talin. HIP12 transcript in brain, heart, kidney, pancreas, and liver, but not in lung or placenta. In ES cell-derived neurons, both HIP1 and HIP12 are highly expressed and distributed throughout the cytoplasm and cell processes with enrichment within the cis-Golgi. In contrast to HIP1, which is toxic in cell culture, HIP12 does not confer toxicity in the same assay systems. HIP12 does not interact with huntingtin but can interact with HIP1, suggesting a potential interaction in vivo that may influence the function of each respective protein.