State involvement in Federal-local grant programs; a case study of the "buying in" approach

The ACIR Library is composed of publications that study the interactions between different levels of government. This document addresses state involvement in federal-local grant programs

Continuity and Change: A Ranking of Key Issues Affecting U.S. Intergovernmental Relations (1995--2005)

Few would dispute that federalism and intergovernmental relations in the United States are dynamic and ever-changing. The actions of the U.S. Congress and executive branch, U.S. Supreme Court cases, state innovations, and political, economic, and international events have direct effects on the relationships between and among governments--often advantaging one governmental level with more power, more discretion, or more responsibilities. It is important for scholars to recognize these actions and trends as they develop and test federalism and intergovernmental relations theories in the United States and as they compare these with federalism events and trends in other federal countries. Copyright 2009, Oxford University Press.

A State response to urban problems: recent experience under the "buying in" approach

The ACIR Library is composed of publications that study the interactions between different levels of government. This document addresses a state response to urban problems

Recruitment of human muscleblind proteins to (CUG)(n) expansions associated with myotonic dystrophy

Myotonic dystrophy (DM1) is an autosomal dominant neuromuscular disorder associated with a (CTG)(n) expansion in the 3′-untranslated region of the DM1 protein kinase (DMPK) gene. To explain disease pathogenesis, the RNA dominance model proposes that the DM1 mutation produces a gain-of-function at the RNA level in which CUG repeats form RNA hairpins that sequester nuclear factors required for proper muscle development and maintenance. Here, we identify the triplet repeat expansion (EXP) RNA-binding proteins as candidate sequestered factors. As predicted by the RNA dominance model, binding of the EXP proteins is specific for dsCUG RNAs and proportional to the size of the triplet repeat expansion. Remarkably, the EXP proteins are homologous to the Drosophila muscleblind proteins required for terminal differentiation of muscle and photoreceptor cells. EXP expression is also activated during mammalian myoblast differentiation, but the EXP proteins accumulate in nuclear foci in DM1 cells. We propose that DM1 disease is caused by aberrant recruitment of the EXP proteins to the DMPK transcript (CUG)(n) expansion