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{{PBB|geneid=2731}}
This is a preview for the new '''MathML rendering mode''' (with SVG fallback), which is availble in production for registered users.
{{enzyme
| Name = glycine dehydrogenase (decarboxylating)
| EC_number = 1.4.4.2
| CAS_number = 37259-67-9
| IUBMB_EC_number = 1/4/4/2
| GO_code = 0004375
| image =
| width =
| caption =
}}
'''Glycine dehydrogenase [decarboxylating], mitochondrial''' also known as '''glycine cleavage system P protein''' or '''glycine decarboxylase''' is an [[enzyme]] that in humans is encoded by the ''GLDC'' [[gene]].<ref name="pmid1993704">{{cite journal | author = Kume A, Koyata H, Sakakibara T, Ishiguro Y, Kure S, Hiraga K | title = The glycine cleavage system. Molecular cloning of the chicken and human glycine decarboxylase cDNAs and some characteristics involved in the deduced protein structures | journal = J Biol Chem | volume = 266 | issue = 5 | pages = 3323–9 | year = 1991 | month = Mar | pmid = 1993704 | pmc =  | doi =  }}</ref><ref name="pmid1996985">{{cite journal | author = Kure S, Narisawa K, Tada K | title = Structural and expression analyses of normal and mutant mRNA encoding glycine decarboxylase: three-base deletion in mRNA causes nonketotic hyperglycinemia | journal = Biochem Biophys Res Commun | volume = 174 | issue = 3 | pages = 1176–82 | year = 1991 | month = Mar | pmid = 1996985 | pmc =  | doi =10.1016/0006-291X(91)91545-N  }}</ref><ref name="entrez">{{cite web | title = Entrez Gene: GLDC glycine dehydrogenase (decarboxylating)| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=2731| accessdate = }}</ref>


== Reaction ==
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Glycine dehydrogenase (decarboxylating) ({{EC number|1.4.4.2}}) is an [[enzyme]] that [[catalysis|catalyzes]] the following [[chemical reaction]]:
Registered users will be able to choose between the following three rendering modes:  


:glycine + H-protein-lipoyllysine <math>\rightleftharpoons</math> H-protein-S-aminomethyldihydrolipoyllysine + CO<sub>2</sub>
'''MathML'''
:<math forcemathmode="mathml">E=mc^2</math>


Thus, the two [[substrate (biochemistry)|substrates]] of this enzyme are [[glycine]] and H-protein-lipoyllysine, whereas its two [[product (chemistry)|products]] are H-protein-S-aminomethyldihydrolipoyllysine and [[carbon dioxide|CO<sub>2</sub>]].
<!--'''PNG'''  (currently default in production)
:<math forcemathmode="png">E=mc^2</math>


This enzyme belongs to the family of [[oxidoreductase]]s, specifically those acting on the CH-NH2 group of donors with a disulfide as acceptor.  This enzyme participates in glycine, serine and threonine metabolism.  It employs one [[cofactor (biochemistry)|cofactor]], [[pyridoxal phosphate]].
'''source'''
:<math forcemathmode="source">E=mc^2</math> -->


== Function ==
<span style="color: red">Follow this [https://en.wikipedia.org/wiki/Special:Preferences#mw-prefsection-rendering link] to change your Math rendering settings.</span> You can also add a [https://en.wikipedia.org/wiki/Special:Preferences#mw-prefsection-rendering-skin Custom CSS] to force the MathML/SVG rendering or select different font families. See [https://www.mediawiki.org/wiki/Extension:Math#CSS_for_the_MathML_with_SVG_fallback_mode these examples].


Glycine decarboxylase is a P-protein of the [[glycine cleavage system]] in [[eukaryotes]].  The glycine cleavage system catalyzes the degradation of glycine. The P protein binds the alpha-amino group of glycine through its [[pyridoxal phosphate]] cofactor. Carbon dioxide is released and the remaining methylamine moiety is then transferred to the lipoamide cofactor of the H protein.
==Demos==


Degradation of glycine is brought about by the glycine cleavage system, which is composed of four mitochondrial protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase).<ref name="entrez"/>
Here are some [https://commons.wikimedia.org/w/index.php?title=Special:ListFiles/Frederic.wang demos]:


== Clinical significance ==


[[D-Glyceric acidemia|Glycine encephalopathy]] may be due to a defect in any one of these enzymes.<ref name="entrez">{{cite web | title = Entrez Gene: GLDC glycine dehydrogenase (decarboxylating)| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=2731| accessdate = }}</ref>
* accessibility:
** Safari + VoiceOver: [https://commons.wikimedia.org/wiki/File:VoiceOver-Mac-Safari.ogv video only], [[File:Voiceover-mathml-example-1.wav|thumb|Voiceover-mathml-example-1]], [[File:Voiceover-mathml-example-2.wav|thumb|Voiceover-mathml-example-2]], [[File:Voiceover-mathml-example-3.wav|thumb|Voiceover-mathml-example-3]], [[File:Voiceover-mathml-example-4.wav|thumb|Voiceover-mathml-example-4]], [[File:Voiceover-mathml-example-5.wav|thumb|Voiceover-mathml-example-5]], [[File:Voiceover-mathml-example-6.wav|thumb|Voiceover-mathml-example-6]], [[File:Voiceover-mathml-example-7.wav|thumb|Voiceover-mathml-example-7]]
** [https://commons.wikimedia.org/wiki/File:MathPlayer-Audio-Windows7-InternetExplorer.ogg Internet Explorer + MathPlayer (audio)]
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** [https://commons.wikimedia.org/wiki/File:MathPlayer-Braille-Windows7-InternetExplorer.png Internet Explorer + MathPlayer (braille)]
** NVDA+MathPlayer: [[File:Nvda-mathml-example-1.wav|thumb|Nvda-mathml-example-1]], [[File:Nvda-mathml-example-2.wav|thumb|Nvda-mathml-example-2]], [[File:Nvda-mathml-example-3.wav|thumb|Nvda-mathml-example-3]], [[File:Nvda-mathml-example-4.wav|thumb|Nvda-mathml-example-4]], [[File:Nvda-mathml-example-5.wav|thumb|Nvda-mathml-example-5]], [[File:Nvda-mathml-example-6.wav|thumb|Nvda-mathml-example-6]], [[File:Nvda-mathml-example-7.wav|thumb|Nvda-mathml-example-7]].
** Orca: There is ongoing work, but no support at all at the moment [[File:Orca-mathml-example-1.wav|thumb|Orca-mathml-example-1]], [[File:Orca-mathml-example-2.wav|thumb|Orca-mathml-example-2]], [[File:Orca-mathml-example-3.wav|thumb|Orca-mathml-example-3]], [[File:Orca-mathml-example-4.wav|thumb|Orca-mathml-example-4]], [[File:Orca-mathml-example-5.wav|thumb|Orca-mathml-example-5]], [[File:Orca-mathml-example-6.wav|thumb|Orca-mathml-example-6]], [[File:Orca-mathml-example-7.wav|thumb|Orca-mathml-example-7]].
** From our testing, ChromeVox and JAWS are not able to read the formulas generated by the MathML mode.


==References==
==Test pages ==
{{reflist}}


==Further reading==
To test the '''MathML''', '''PNG''', and '''source''' rendering modes, please go to one of the following test pages:
{{refbegin | 2}}
*[[Displaystyle]]
* {{cite journal | author = Hiraga K, Kikuchi G | date = 1980 | title = The mitochondrial glycine cleavage system. Functional association of glycine decarboxylase and aminomethyl carrier protein | journal = J. Biol. Chem.  | volume = 255 | pages = 11671&ndash;6  | pmid = 7440563 | issue = 24 }}
*[[MathAxisAlignment]]
* {{cite journal | author = Perham RN | date = 2000 | title = Swinging arms and swinging domains in multifunctional enzymes: catalytic machines for multistep reactions | journal = Annu. Rev. Biochem.  | volume = 69 | pages = 961&ndash;1004  | pmid = 10966480 | doi = 10.1146/annurev.biochem.69.1.961 }}
*[[Styling]]
* {{cite journal | author = Broadwater JA, Haas JA, Fox BG, Booker SJ | date = 2005 | title = Expression, purification, and physical characterization of Escherichia coli lipoyl(octanoyl)transferase | journal = Protein. Expr. Purif.  | volume = 39 | pages = 269&ndash;82  | pmid = 15642479 | issue = 2 | doi = 10.1016/j.pep.2004.10.021 }}*{{cite journal  | author=Applegarth DA, Toone JR |title=Nonketotic hyperglycinemia (glycine encephalopathy): laboratory diagnosis |journal=Mol. Genet. Metab. |volume=74 |issue= 1–2 |pages= 139–46 |year= 2001 |pmid= 11592811 |doi= 10.1006/mgme.2001.3224 }}
*[[Linebreaking]]
*{{cite journal  | author=Kure S, Takayanagi M, Narisawa K, ''et al.'' |title=Identification of a common mutation in Finnish patients with nonketotic hyperglycinemia |journal=J. Clin. Invest. |volume=90 |issue= 1 |pages= 160–4 |year= 1992 |pmid= 1634607 |doi=10.1172/JCI115831  | pmc=443076  }}
*[[Unique Ids]]
*{{cite journal  | author=Sakakibara T, Koyata H, Ishiguro Y, ''et al.'' |title=One of the two genomic copies of the glycine decarboxylase cDNA has been deleted at a 5' region in a patient with nonketotic hyperglycinemia |journal=Biochem. Biophys. Res. Commun. |volume=173 |issue= 3 |pages= 801–6 |year= 1991 |pmid= 2268343 |doi=10.1016/S0006-291X(05)80858-7  }}
*[[Help:Formula]]
*{{cite journal  | author=Burton BK, Pettenati MJ, Block SM, ''et al.'' |title=Nonketotic hyperglycinemia in a patient with the 9p- syndrome |journal=Am. J. Med. Genet. |volume=32 |issue= 4 |pages= 504–5 |year= 1989 |pmid= 2773994 |doi= 10.1002/ajmg.1320320416 }}
*{{cite journal  | author=Hayasaka K, Kochi H, Hiraga K, Kikuchi G |title=Purification and properties of glycine decarboxylase, a component of the glycine cleavage system, from rat liver mitochondria and immunochemical comparison of this enzyme from various sources |journal=J. Biochem. |volume=88 |issue= 4 |pages= 1193–9 |year= 1981 |pmid= 6778858 |doi=  }}
*{{cite journal  | author=Hiraga K, Kochi H, Hayasaka K, ''et al.'' |title=Defective glycine cleavage system in nonketotic hyperglycinemia. Occurrence of a less active glycine decarboxylase and an abnormal aminomethyl carrier protein |journal=J. Clin. Invest. |volume=68 |issue= 2 |pages= 525–34 |year= 1981 |pmid= 6790577 |doi=10.1172/JCI110284  | pmc=370827  }}
*{{cite journal  | author=Takayanagi M, Kure S, Sakata Y, ''et al.'' |title=Human glycine decarboxylase gene (GLDC) and its highly conserved processed pseudogene (psiGLDC): their structure and expression, and the identification of a large deletion in a family with nonketotic hyperglycinemia |journal=Hum. Genet. |volume=106 |issue= 3 |pages= 298–305 |year= 2000 |pmid= 10798358 |doi=10.1007/s004390051041  }}
*{{cite journal  | author=Toone JR, Applegarth DA, Coulter-Mackie MB, James ER |title=Biochemical and molecular investigations of patients with nonketotic hyperglycinemia |journal=Mol. Genet. Metab. |volume=70 |issue= 2 |pages= 116–21 |year= 2000 |pmid= 10873393 |doi= 10.1006/mgme.2000.3000 }}
*{{cite journal  | author=Toone JR, Applegarth DA, Coulter-Mackie MB, James ER |title=Recurrent mutations in P- and T-proteins of the glycine cleavage complex and a novel T-protein mutation (N145I): a strategy for the molecular investigation of patients with nonketotic hyperglycinemia (NKH) |journal=Mol. Genet. Metab. |volume=72 |issue= 4 |pages= 322–5 |year= 2001 |pmid= 11286506 |doi= 10.1006/mgme.2001.3158 }}
*{{cite journal  | author=Kure S, Kojima K, Ichinohe A, ''et al.'' |title=Heterozygous GLDC and GCSH gene mutations in transient neonatal hyperglycinemia |journal=Ann. Neurol. |volume=52 |issue= 5 |pages= 643–6 |year= 2002 |pmid= 12402263 |doi= 10.1002/ana.10367 }}
*{{cite journal  | author=Strausberg RL, Feingold EA, Grouse LH, ''et al.'' |title=Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=99 |issue= 26 |pages= 16899–903 |year= 2003 |pmid= 12477932 |doi= 10.1073/pnas.242603899  | pmc=139241 }}
*{{cite journal  | author=Toone JR, Applegarth DA, Laliberte G |title=Gene Symbol: GLDC. Disease: NKH glycine encephalopathy |journal=Hum. Genet. |volume=113 |issue= 5 |pages= 465 |year= 2003 |pmid= 14552331 |doi=10.1007/s00439-003-1014-5  }}
*{{cite journal  | author=Dinopoulos A, Kure S, Chuck G, ''et al.'' |title=Glycine decarboxylase mutations: a distinctive phenotype of nonketotic hyperglycinemia in adults |journal=Neurology |volume=64 |issue= 7 |pages= 1255–7 |year= 2006 |pmid= 15824356 |doi= 10.1212/01.WNL.0000156800.23776.40 }}
*{{cite journal  | author=Flusser H, Korman SH, Sato K, ''et al.'' |title=Mild glycine encephalopathy (NKH) in a large kindred due to a silent exonic GLDC splice mutation |journal=Neurology |volume=64 |issue= 8 |pages= 1426–30 |year= 2006 |pmid= 15851735 |doi= 10.1212/01.WNL.0000158475.12907.D6 }}
*{{cite journal  | author=Boneh A, Korman SH, Sato K, ''et al.'' |title=A single nucleotide substitution that abolishes the initiator methionine codon of the GLDC gene is prevalent among patients with glycine encephalopathy in Jerusalem |journal=J. Hum. Genet. |volume=50 |issue= 5 |pages= 230–4 |year= 2005 |pmid= 15864413 |doi= 10.1007/s10038-005-0243-y }}
*{{cite journal  | author=Kimura K, Wakamatsu A, Suzuki Y, ''et al.'' |title=Diversification of transcriptional modulation: large-scale identification and characterization of putative alternative promoters of human genes |journal=Genome Res. |volume=16 |issue= 1 |pages= 55–65 |year= 2006 |pmid= 16344560 |doi= 10.1101/gr.4039406  | pmc=1356129 }}
*{{cite journal  | author=Korman SH, Wexler ID, Gutman A, ''et al.'' |title=Treatment from birth of nonketotic hyperglycinemia due to a novel GLDC mutation |journal=Ann. Neurol. |volume=59 |issue= 2 |pages= 411–5 |year= 2006 |pmid= 16404748 |doi= 10.1002/ana.20759 }}
}}
{{refend}}


{{1.4-enzyme-stub}}
*[[Inputtypes|Inputtypes (private Wikis only)]]
 
*[[Url2Image|Url2Image (private Wikis only)]]
[[Category:EC 1.4.4]]
==Bug reporting==
[[Category:Pyridoxal phosphate enzymes]]
If you find any bugs, please report them at [https://bugzilla.wikimedia.org/enter_bug.cgi?product=MediaWiki%20extensions&component=Math&version=master&short_desc=Math-preview%20rendering%20problem Bugzilla], or write an email to math_bugs (at) ckurs (dot) de .
[[Category:Enzymes of known structure]]
 
[[es:Glicina deshidrogenasa (descarboxilante)]]
[[it:Glicina deidrogenasi (decarbossilante)]]
[[ja:グリシンデヒドロゲナーゼ (脱炭酸)]]

Latest revision as of 22:52, 15 September 2019

This is a preview for the new MathML rendering mode (with SVG fallback), which is availble in production for registered users.

If you would like use the MathML rendering mode, you need a wikipedia user account that can be registered here [[1]]

  • Only registered users will be able to execute this rendering mode.
  • Note: you need not enter a email address (nor any other private information). Please do not use a password that you use elsewhere.

Registered users will be able to choose between the following three rendering modes:

MathML

E=mc2


Follow this link to change your Math rendering settings. You can also add a Custom CSS to force the MathML/SVG rendering or select different font families. See these examples.

Demos

Here are some demos:


Test pages

To test the MathML, PNG, and source rendering modes, please go to one of the following test pages:

Bug reporting

If you find any bugs, please report them at Bugzilla, or write an email to math_bugs (at) ckurs (dot) de .

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