Crystal structure of human branched-chain α-ketoacid dehydrogenase and the molecular basis of...

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Crystal structure of human branched-chain α-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease Arnthor Ævarsson, Jacinta L Chuang, R Max Wynn, Stewart Turley, David T Chuang, Wim GJ Hol Structure Volume 8, Issue 3, Pages 277-291 (March 2000) DOI: 10.1016/S0969-2126(00)00105-2

Transcript of Crystal structure of human branched-chain α-ketoacid dehydrogenase and the molecular basis of...

Page 1: Crystal structure of human branched-chain α-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease.

Crystal structure of human branched-chain α-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease 

Arnthor Ævarsson, Jacinta L Chuang, R Max Wynn, Stewart Turley, David T Chuang, Wim GJ Hol 

Structure Volume 8, Issue 3, Pages 277-291 (March 2000)

DOI: 10.1016/S0969-2126(00)00105-2

Page 2: Crystal structure of human branched-chain α-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease.

Figure 1

Structure 2000 8, 277-291DOI: (10.1016/S0969-2126(00)00105-2)

Page 3: Crystal structure of human branched-chain α-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease.

Figure 2

Structure 2000 8, 277-291DOI: (10.1016/S0969-2126(00)00105-2)

Page 4: Crystal structure of human branched-chain α-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease.

Figure 3

Structure 2000 8, 277-291DOI: (10.1016/S0969-2126(00)00105-2)

Page 5: Crystal structure of human branched-chain α-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease.

Figure 4

Structure 2000 8, 277-291DOI: (10.1016/S0969-2126(00)00105-2)

Page 6: Crystal structure of human branched-chain α-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease.

Figure 5

Structure 2000 8, 277-291DOI: (10.1016/S0969-2126(00)00105-2)

Page 7: Crystal structure of human branched-chain α-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease.

Figure 6

Structure 2000 8, 277-291DOI: (10.1016/S0969-2126(00)00105-2)

Page 8: Crystal structure of human branched-chain α-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease.

Figure 7

Structure 2000 8, 277-291DOI: (10.1016/S0969-2126(00)00105-2)

Page 9: Crystal structure of human branched-chain α-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease.

Figure 8

Structure 2000 8, 277-291DOI: (10.1016/S0969-2126(00)00105-2)


The purpose of experimentation was to determine which carbon source - glucose, glycerol, or maple syrup - caused Bacillus megaterium to produce the most.

The purpose of experimentation was to determine which carbon source - glucose, glycerol, or maple syrup - caused Bacillus megaterium to produce the most.

PotentialApplicationsofImmobilized β-Galactosidasein ...downloads.hindawi.com/journals/er/2010/473137.pdf · into a very useful product like sweet syrup, which can be used in various

PotentialApplicationsofImmobilized β-Galactosidasein ...downloads.hindawi.com/journals/er/2010/473137.pdf · into a very useful product like sweet syrup, which can be used in various

CS206 --- Electronic Commercedata.science.uoit.ca/wp-content/uploads/2014/07/DS_2_relational... · BarInfo( bar, beer, price, addr ) Joe’s Bud 2.50 Maple St. Joe’s Milller 2.75

CS206 --- Electronic Commercedata.science.uoit.ca/wp-content/uploads/2014/07/DS_2_relational... · BarInfo( bar, beer, price, addr ) Joe’s Bud 2.50 Maple St. Joe’s Milller 2.75

BRANCHED AND CROSSLINKED RESORBABLE POLYMERS BASED …lib.tkk.fi/Diss/2003/isbn9512268418/isbn9512268418.pdf · BRANCHED AND CROSSLINKED RESORBABLE POLYMERS ... PREFACE This work

BRANCHED AND CROSSLINKED RESORBABLE POLYMERS BASED …lib.tkk.fi/Diss/2003/isbn9512268418/isbn9512268418.pdf · BRANCHED AND CROSSLINKED RESORBABLE POLYMERS ... PREFACE This work

Highly Branched Poly(α-Methylene-γ-Butyrolactone) … · P. Binda et al. DOI: 10.4236/ojpchem.2017.74006 77 Open Journal of Polymer Chemistry The best and most reliable method of

Highly Branched Poly(α-Methylene-γ-Butyrolactone) … · P. Binda et al. DOI: 10.4236/ojpchem.2017.74006 77 Open Journal of Polymer Chemistry The best and most reliable method of

Rheological properties of sweet potato starch-date syrup gel · the effect of monosaccharides and high Brix date syrup on sweet potato starch gelatinization has not been done to the

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Direct Asymmetric Amination of α Branched Cyclic … of α-substituted cyclic ketones catalyzed by a chiral phosphoric ... block in organic synthesis. ... here we report the direct

Direct Asymmetric Amination of α Branched Cyclic … of α-substituted cyclic ketones catalyzed by a chiral phosphoric ... block in organic synthesis. ... here we report the direct

Thissio1 μπάλα παγωτό με σαντιγύ και σιρόπι σε φωλιά από μπισκότo 3.80€ 1 ice cream scoop with whipped cream and syrup in a biscuit“nest”

Thissio1 μπάλα παγωτό με σαντιγύ και σιρόπι σε φωλιά από μπισκότo 3.80€ 1 ice cream scoop with whipped cream and syrup in a biscuit“nest”

Branched Covers between Surfaces - uniroma1.it

Branched Covers between Surfaces - uniroma1.it

Maple syrup urine disease

Maple syrup urine disease

Characterization of Branched Covers with Simplicial Branch ...eprywes/Branched_Cover_Slides.pdfcover. Theorem (Sto low) Let f : S2!bCbe a branched cover. Then there exists a homeomorphism

Characterization of Branched Covers with Simplicial Branch ...eprywes/Branched_Cover_Slides.pdfcover. Theorem (Sto low) Let f : S2!bCbe a branched cover. Then there exists a homeomorphism

garsia/lectures/FinalMontTalk.pdf · Proceeding by the naive approach Using available symmetric function (MAPLE) packages we get W 5 (q)= 1 + 5q 2 + q 3 + 36q 4 + 15q 5 + 228q 6 +

garsia/lectures/FinalMontTalk.pdf · Proceeding by the naive approach Using available symmetric function (MAPLE) packages we get W 5 (q)= 1 + 5q 2 + q 3 + 36q 4 + 15q 5 + 228q 6 +

Ivyspring International Publisher TheranosticsFA metabolism, the regulatory role of amino acid metabolism in I/R process remains largely unknown. Branched chain amino acids (BCAA),

Ivyspring International Publisher TheranosticsFA metabolism, the regulatory role of amino acid metabolism in I/R process remains largely unknown. Branched chain amino acids (BCAA),

013 - 034 Kefalaio 1 · Το ακόλουθο υλικό έχει αναπτυχθεί χρησιμοποιώντας το Maple στην έκδοση 11 σε λειτουργικό

013 - 034 Kefalaio 1 · Το ακόλουθο υλικό έχει αναπτυχθεί χρησιμοποιώντας το Maple στην έκδοση 11 σε λειτουργικό

Analysis and Purification of RNAi and siRNA...Analysis and Purification of RNAi and siRNA Sean M. McCarthy, Martin Gilar Waters Corporation, 34 Maple Street, Milford, MA 01757 OVERVIEW

Analysis and Purification of RNAi and siRNA...Analysis and Purification of RNAi and siRNA Sean M. McCarthy, Martin Gilar Waters Corporation, 34 Maple Street, Milford, MA 01757 OVERVIEW

Μια Εισαγωγή στη MAPLE · Γεωργίου Εισαγωγή στη maple 4 > 2^72; (2/5)^48; 3^(1/2); ... δυνατότητα είναι ιδιαίτερα χρήσιμη

Μια Εισαγωγή στη MAPLE · Γεωργίου Εισαγωγή στη maple 4 > 2^72; (2/5)^48; 3^(1/2); ... δυνατότητα είναι ιδιαίτερα χρήσιμη

013 - 034 Kefalaio 1 · 2017. 2. 20. · Στο περιβάλλον Standard Maple διαθέτουμε επιπλέον παλέτες και συντομεύσεις ό-πως

013 - 034 Kefalaio 1 · 2017. 2. 20. · Στο περιβάλλον Standard Maple διαθέτουμε επιπλέον παλέτες και συντομεύσεις ό-πως

Maple Syrup Urine Disease - University of Wisconsin-Eau Claire

Maple Syrup Urine Disease - University of Wisconsin-Eau Claire

Highly Branched Poly(α-Methylene-γ-Butyrolactone) from …file.scirp.org/pdf/OJPChem_2017112914172525.pdf · 2017-12-01 · ... (3.00 g, 0.013 mol), and L-valinol [(S)-(+)-2-Amino-3-methyl-1-butanol]

Highly Branched Poly(α-Methylene-γ-Butyrolactone) from …file.scirp.org/pdf/OJPChem_2017112914172525.pdf · 2017-12-01 · ... (3.00 g, 0.013 mol), and L-valinol [(S)-(+)-2-Amino-3-methyl-1-butanol]

Study of a 2-branched (1→→3)-ββ-D-glucan from ... · solution a fairly rough morphology with a high density of ... 2.5 AFM Imaging ... ropy strain of Lactobacillus suebicus

Study of a 2-branched (1→→3)-ββ-D-glucan from ... · solution a fairly rough morphology with a high density of ... 2.5 AFM Imaging ... ropy strain of Lactobacillus suebicus