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The Pax-6 gene encodes a DNA-binding transcription factor essential to normal development of the mammalian eye. We have found that in the chick embryo, the Pax-6 gene is first expressed in a crescent-shaped region of future head ectoderm that adjoins the anterior margin of the early neural plate. As development proceeds, this region of Pax-6-positive(More)
Development or regeneration of axons in several systems is accompanied by 20-100-fold increases in the synthesis of an acidic, axonally transported membrane protein with an apparent molecular weight of 43-50,000 (Benowitz and Lewis, 1983; Skene and Willard, 1981a, b), which we designate GAP-43. We have proposed that some step(s) in axon growth require(More)
Growth cones are specialized structures that form the distal tips of growing axons. During both normal development of the nervous system and regeneration of injured nerves, growth cones are essential for elongation and guidance of growing axons. Developmental and regenerative axon growth is frequently accompanied by elevated synthesis of a protein(More)
Nerve regeneration and developmental outgrowth of axons are both correlated with increased synthesis of an axonal membrane protein designated GAP-43. Phosphorylation of an apparently identical protein, present at lower abundance in adult brains, has been correlated with long-term potentiation, a form of synaptic plasticity. We have now isolated a cDNA clone(More)
We applied functional magnetic resonance imaging (FMRI) to map the somatotopic organization of the primary motor cortex using voluntary movements of the hand, arm, and foot. Eight right-handed healthy subjects performed self-paced, repetitive, flexion/extension movements of the limbs while undergoing echo-planar imaging. Four subjects performed movements of(More)
Linear scleroderma represents a unique form of localized scleroderma that primarily affects the pediatric population, with 67% of patients diagnosed before 18 years of age. When linear scleroderma occurs on the head, it is referred to as linear scleroderma en coup de sabre, given the resemblance of the skin lesions to the stroke of a sabre. Here we describe(More)
A number of familial syndromes of bilateral polymicrogyria (PMG) have been described, but reported unilateral PMG cases have generally been sporadic. The authors identified four families in which unilateral right-sided PMG on MRI was present in more than one individual, with pathologic confirmation in one. Core clinical features included contralateral(More)
To determine if there is abnormal phenylalanine and biopterin metabolism in patients with dopa-responsive dystonia (DRD), we measured plasma levels of phenylalanine, tyrosine, biopterin, and neopterin at baseline, and 1, 2, 4, and 6 hours after an oral phenylalanine load (100 mg/kg). Seven adults with DRD, two severely affected children with DRD, and nine(More)
Intramuscular injections of HRP were used to map the spinal cord location of motoneurons innervating wing and shoulder girdle muscles in newly hatched chicks. Motor pools are grouped in the lateral motor column in relationship to embryonic origin of the muscles: muscles derived from the ventral muscle mass are innervated by medially lying motor pools, while(More)