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One of the most challenging concepts to grasp when using ActionScript is the meaning of the this keyword The this keyword is relative; its meaning changes, depending on where it is in the code It refers to the object where the this keyword resides For example, on the Main Timeline, the this keyword represents the Main Timeline Inside a movie clip, the this keyword represents that movie clip If the this keyword is inside an event handler attached to a movie clip, the this keyword represents the movie clip to which the event handler is attached
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Approximately 20% of people with CMT1 have CMT1B, which is caused by mutations in the MPZ gene located on chromosome 1q22 2336 38 CMT1B is for the most part clinically, electrophysiologically, and histologically indistinguishable from CMT1A However, patients with MPZ mutation are more likely to have more axonal physiology on NCS than those patients with PMP-22 mutations Also, CMT associated with Adies pupils are more common in patients with MPZ mutations MPZ is an integral myelin protein and accounts for more than half of the myelin protein in peripheral nerves It is a member of the immunoglobulin superfamily and consists of an extracellular immunoglobulin-like domain, a transmembrane domain, and a cytoplasmic domain1 MPZ localizes to the tight compact regions of myelin, where it may play a role in maintaining tight compaction by forming links between adjacent myelin layers Nerve biopsies in people with CMT1B reveal abnormalities similar to that noted in CMT1A However, occasionally tomaculae and uncompacted myelin are apparent, which are not typically seen on nerve biopsy in CMT1A4,12,39 Immunohistochemistry and ultrastructural studies on nerve biopsy specimens may demonstrate decreased expression of MPZ protein40 Some mutations in the MPZ gene have been associated with a severe demyelinating CMT3 phenotype, while others are associated with NCS suggestive of an axonopathy or CMT2 The speci c location of the mutations in the MPZ gene and how these affect the function of the myelin protein probably account for the phenotypic heterogeneity
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CMT1D44 ERG2 is believed to be a transcription factor that binds DNA through three zinc nger domains and likely has an important action in regulating myelin genes in Schwann cells CMT1D accounts for <1% of molecularde ned cases of CMT1
Before we started our discussion on the input buffer, main() had just called printf() to prompt for the user s first name:
This refers to kinships with CMT1 associated with deafness It has been demonstrated to be allelic to CMT1A and caused by point mutations in PMP-22 gene5
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In order to animate, you need to make changes in art This is where keyframes come in A keyframe is a special kind of frame you can use to create or change art You are already familiar with keyframes All the drawing you have done so far has been on keyframes In fact, you can only draw on keyframes To create a keyframe, click in the area of the Timeline where you would like to create a keyframe and choose Insert | Timeline | Keyframe, or use the keyboard shortcut F6 This is another shortcut I recommend memorizing, since you will be creating keyframes often When you create a keyframe, you will see a black dot inside the frame The black dot inside the frame means there is art in that keyframe (see Figure 1-7)
Mutations in the neuro lament light chain (NF-L) gene located on chromosome 8p13 21 are usually associated with low-amplitude CMAPs and normal or only slightly slow NCVs and thus are often categorized as an axonal form of CMT (CMT2E) However, some cases have been reported with motor NCVs in the mid-twenties and thus have been classi ed as a CMT1F45,46
printf( "Type your first name, please: " );
Figure 9 5 The origin of Charcot Marie Tooth syndrome type 1A (CMT1A) and hereditary neuropathy with liability to pressure palsies (HNPP) Relative locations of the CMT1A repeat sequence (CMT1A-REP) and the peripheral myelin protein-22 gene (PMP-22) in chromosome 17p112 12 are shown Normally only one copy of the PMP-22 gene is present on each chromosome However, misalignment of the CMT1A-REP sequence during meiosis may lead to tandem duplication in CMT1A (three copies of the PMP-22 gene) or an interstitial deletion in HNPP (one copy of PMP-22 gene) Rare cases inheriting duplicated copies of PMP-22 gene (four copies) in both chromosomes are associated with a severe CMT1 clinical phenotype (With permission from Lupski JR, Chance PF, Garcia CA Inherited primary peripheral neuropathies: Molecular genetics and clinical implications of CMT1 A and HNPP JAMA 1993;270(19):2326 2330, Fig 1, p 2327)
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