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MAT deficiency

Beta-ketothiolase deficiency is an uncommon inherited disorder in which the body cannot properly process the amino acid isolecine or the products of lipid breakdown. The condition is inherited in an autosomal recessive pattern and is extremely rare having only been reported in 50 to 60 individuals throughout the world. more...

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The typical age of onset for this disorder is between 6 months and 24 months. The signs and symptoms of beta-ketothiolase deficiency include vomiting, dehydration, trouble breathing, extreme tiredness, and occasionally convulsions. These episodes are called ketoacidotic attacks and can sometimes lead to coma. Attacks occur when compounds called organic acids (which are formed as products of amino acid and fat breakdown) build up to toxic levels in the blood. These attacks are often triggered by an infection, fasting (not eating), or in some cases, other types of stress.

Mutations in the ACAT1 gene cause beta-ketothiolase deficiency. The enzyme made by the ACAT1 gene plays an essential role in breaking down proteins and fats in the diet. Specifically, the enzyme is responsible for processing isoleucine, an amino acid that is part of many proteins. This enzyme also processes ketones, which are produced during the breakdown of fats. If a mutation in the ACAT1 gene reduces or eliminates the activity of this enzyme, the body is unable to process isoleucine and ketones properly. As a result, harmful compounds can build up and cause the blood to become too acidic (ketoacidosis), which impairs tissue function, especially in the central nervous system.

This article incorporates public domain text from The U.S. National Library of Medicine

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Being there: Michael Fried on two pictures by Jeff Wall
From ArtForum, 9/1/04 by Michael Fried

THINKING ABOUT Jeff Wall's most recent exhibition in New York, a show of light-box pictures at Marian Goodman Gallery last spring, has led me to reflect on the more philosophical or, say, ontological turn his work has taken during the past four or five years. The central image in the show was Fieldwork. Excavation of the floor of a dwelling in a former Sto:Io nation village, Greenwood Island, Hope, University of California at Los Angeles, working with Riley Lewis of the Sto:Io band, 2003. For all the information the title provides, it doesn't quite say everything. The picture offers us a largely downward view into and across a clearing in a forest where two men are at work. One of the men is seated cross-legged on the ground (actually, he sits on a wooden "mat") before a squarish hole, perhaps a foot and a half deep, which even without the title is recognizable as the product of meticulous excavation. A second man, Lewis, stands about fifteen feet away, looking on as Graesch concentrates on his task (taking soil samples, apparently). The clearing itself gives the impression of being partly man-made: Certainly, some preliminary ground preparation and digging have taken place, and the site has been tagged with bits of orange plastic that make vivid color accents against the deep green of foliage. Orange and yellow plastic pails and a blue dustpan are also crucial to the pictorial effect, as are the wooden mats that radiate outward from the hole for the practical purpose of keeping workers from disturbing the ground but which here serve the pictorial function of directing the viewer's attention to the central motif. There is also a second squarish hole, farther away and to the left of the first, partly obscured by the seated anthropologist. The overall effect is calm, contemplative, absorptive, even as the not inconsiderable distance of the viewer (who is, in the first place, the photographer) precludes all possibility of identification with the two men.

As anyone who has been following Wall's art will have recognized, Fieldwork is an example of the pictorial mode he calls "near documentary," which he has described as "claim[ing] to be a plausible account of, or a report on, what the events depicted are like, or were like, when they passed without being photographed." This description exactly characterizes another recent work, A woman with a covered tray, 2003, that is based on an incident Wall actually saw but which he made in collaboration with the young woman depicted in it through a process of trial and error (having the young woman walk toward the camera, then having her walk away from it, taking innumerable shots in search of just the right image). The final image also required the subsequent putting together of the two basic elements it comprises, the young woman and the house-and-garden setting, with the aid of computer technology. Fieldwork, on the other hand, is not a reenactment of an event but rather a depiction--I don't quite wish to say a straight photograph--of a scene actually taking place just as it appears to us (maybe "near near documentary" is how we should think about it). Wall spent three weeks at the site, first erecting a scaffolding from which to take pictures and then taking a large number of photographs, the idea being that the men excavating the holes would become so accustomed to his presence as to eventually discount it and behave naturally. There is no reason to doubt that this is more or less what happened, but of course the viewer cannot but be conscious of the photographer's elevated viewpoint, and more broadly of the brilliant artifactual character of the light-box image, including the usual horizontal line dividing the upper and lower sections of the transparency.

[ILLUSTRATION OMITTED]

This is not said critically. On the contrary, the essence of Wall's "near documentary" (and now "near near documentary") approach consists precisely in the overt tension between the absorptiveness and antitheatricality of his chosen motifs and the artifactuality and to-be-seen-ness (I'm stopping just short of saying "theatricality") of his autograph means of presentation (perhaps we can think of this as a tension between representation and presentation). In an important sense, of course, such a tension marked absorptive painting from the first: The primordial convention (as I have called it elsewhere) that paintings are made to be beheld could not be defeated by French painters of the eighteenth and nineteenth centuries--only temporarily neutralized, put out of mind. But in Wall's work, as in other recent photography (I will mention only Thomas Struth, Andreas Gursky, Beat Streuli, and Philip-Lorca diCorcia), something subtly different happens: An antitheatrical impulse, keyed in Wall's case to a documentary look and an absorptive dramaturgy, coexists with a foregrounding of the apparatus and a radical acknowledgment of the roles of both photographer and beholder. All this marks a dramatic new turning in the relations between theatricality and antitheatricality, not just as the conflict between the two was analyzed at its inception in the late 1750s and '60s by Denis Diderot, but also as it was portrayed at a much later point in its trajectory in "Art and Objecthood," an essay whose relevance to the present state of photographic art remains to be made out (that will be the task of a book I am currently writing). Another feature of Fieldwork worth commenting on is the equally sharp focus across the whole of its expanse. To Wall's thinking, the resulting image is closer in feeling to ordinary vision, in which we are normally never conscious of objects nearer or farther away being blurred relative to those we are focusing on, as is often the case in photographs. But there is no equivalent in ordinary vision to such simultaneous evenness of focus across objects at varying distances from the viewer, which is to say that in this respect, too, Fieldwork's artifactual character is subtly highlighted. (To achieve such evenness of focus requires the use of a computer to combine more than one image, which is why I didn't characterize the picture as a whole as an instance of straight photography.)

[ILLUSTRATION OMITTED]

It is in terms of subject matter that Fieldwork reveals the depth of its ontological interests. Consider the principal hole with its multiple strata, each of which represents a particular period of time and a particular material reality. (One of the strata, partway down, may be what is left of the roof of a dwelling that once stood at that spot.) One commonplace idea about photography is that it depicts surfaces and traces on surfaces. No doubt Wall would agree: A wall in a former bakery, 2003, another work at Marian Goodman, comprises nothing else. But what we find in Fieldwork is a thematization of the thickness or, more precisely, the layeredness of the world--by which I mean the way material traces (so many "surfaces"), laid down day by day in earlier epochs, are part of the very substance of reality. There is also the thematization of a certain patient labor of recovery, which we are allowed to witness only from a respectful distance and with which Wall doubtless wishes to associate his art. The phrase "day by day" links Fieldwork with another of Wall's great themes, the everyday, which in his work has evolved from a primary concern with modern life (as in Baudelaire's title "The Painter of Modern Life") to an almost Wittgensteinian preoccupation with the everyday as such. (This, too, is a large subject that points beyond the scope of the present remarks.) In Fieldwork, that preoccupation receives perhaps its most deeply pondered treatment yet in Wall's art. The seeming obliviousness of the two men to the fact of being beheld is itself a hallmark of depictions of life lived in the mode of the everyday (a Wittgensteinian claim that will require further proof), even as the aim of their absorbed activity is the systematic excavation and analysis of the layered remains of a continuum of previous everydays.

Layeredness of a related sort is also in play in Staining bench, furniture manufacturer's, Vancouver, 2003--a work presented with Fieldwork in Wall's New York show--which I want experimentally to read in terms of the Heideggerian notion of the priority of Dasein's engagement with practical tasks over any other mode of relation in the world. Briefly: In Sein und Zeit (1927) Heidegger stands opposed to the notion that, primordially, Dasein confronts a world of objects in and of themselves (in what he calls the mode of "present-at-hand"). Rather, he conceives of Dasein as continually "absorbed"--the German infinitive is aufgehen--in practical activity, which is to say, as continually putting things to use for particular purposes (such things are "ready-to-hand," in his idiom). Only when that relationship is suspended, either because a piece of equipment breaks down or for some comparable reason, does Dasein enter "the sole remaining mode of Being-in, the mode of just tarrying alongside.... This kind of Being towards the world is one which lets us encounter entities within-the-world purely in the way they look, just that." We might think that encountering entities "within-the-world" purely in the way they look is the inevitable fate of photography, or at least of straight photography. But in Staining bench, as in other recent pictures by Wall (After Invisible Man by Ralph Ellison, the Preface, 1999-2001, is perhaps the most impressive instance of this), the artist seems actively to have sought to depict some portion of the world (in the latter case, the Invisible Man's underground refuge) as distinctly other than "a context of extended Things which are just present-to-hand and no more." Thus, in Staining bench, the neatly arranged cans of stain, the brushes and thin painter's gloves resting on the lid of the nearest can, and the wooden stirrer leaning against that can are, although not shown in actual use, not presented as marked by a "deficiency in our having-to-do with the world concernfully." Rather, they are depicted in a way that thematizes both the uses to which they have been put and the work-world--Heidegger would say the "region"--within which they have been employed (and will be again, we do not doubt). The image itself, at three feet by less than four feet, is not large. But it is so remarkably replete, so richly tactile, so densely layered with material traces of practical human activity (the gloves, once we notice them, seem almost like shed skin, while the table cover impregnated with stain seems sticky to the touch) that it might be said at once to confirm and to escape Heidegger's categories. It is as if the photograph represents equipment, things in the mode of readiness-to-hand (or potential readiness-to-hand; I am improvising here)--but not for us.

Michael Fried is J.R. Herbert Boone Professor of Humanities and director of the Humanities Center at the Johns Hopkins University. He is the author of numerous books and articles, including Art and Objecthood: Essays and Reviews and Menzel's Realism: Art and Embodiment in Nineteenth-Century Berlin. His most recent publication is The Next Bend in the Road (University of Chicago Press, 2004), a book of poems.

COPYRIGHT 2004 Artforum International Magazine, Inc.
COPYRIGHT 2004 Gale Group

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