Protein basic information
| LiverAtlas Protein ID |
HuLPr45229 |
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Uniprot ID |
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Uniprot Acc |
Q9UL12;B2RMR5;B7ZLT6;Q5SYV0;Q9Y280;Q9Y2Y3; |
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Protein name |
Sarcosine dehydrogenase, mitochondrial |
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Comment |
CATALYTIC ACTIVITY:Sarcosine + acceptor + H(2)O = glycine + formaldehyde + reduced acceptor.||COFACTOR:Binds 1 FAD covalently per monomer (By similarity).||PATHWAY:Amine and polyamine degradation; sarcosine degradation; formaldehyde and glycine from sarcosine:step 1/1.||SUBCELLULAR LOCATION:Mitochondrion matrix (By similarity).||DISEASE:Defects in SARDH are a cause of sarcosinemia (SAR) [MIM:268900]. Sarcosinemia is a metabolic disorder characterized by an increased concentration of sarcosine in plasma and urine due to sarcosine dehydrogenase deficiency. Prevalence has been estimated at 1:28'000 to 1:350'000 in newborn screening programs. Sarcosinemia is most probably a benign condition without significant clinical problems. It is transmitted in an autosomal recessive manner.||SIMILARITY:Belongs to the GcvT family.||SEQUENCE CAUTION:Sequence=AAD33412.1; Type=Frameshift; Positions=867; |
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Subcellular localization |
Mitochondrion matrix(By similarity). |
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Gene name |
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Related liver disease name |
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Protein sequence
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MASLSRALRVAAAHPRQSPTRGMGPCNLSSAAGPTAEKSV PYQRTLKEGQGTSVVAQGPSRPLPSTANVVVIGGGSLGC QTLYHLAKLGMSGAVLLERERLTSGTTWHTAGLLWQLRP SDVEVELLAHTRRVVSRELEEETGLHTGWIQNGGLFIAS NRQRLDEYKRLMSLGKAYGVESHVLSPAETKTLYPLMNV DDLYGTLYVPHDGTMDPAGTCTTLARAASARGAQVIENC PVTGIRVWTDDFGVRRVAGVETQHGSIQTPCVVNCAGVW ASAVGRMAGVKVPLVAMHHAYVVTERIEGIQNMPNVRDH DASVYLRLQGDALSVGGYEANPIFWEEVSDKFAFGLFDL DWEVFTQHIEGAINRVPVLEKTGIKSTVCGPESFTPDHK PLMGEAPELRGFFLGCGFNSAGMMLGGGCGQELAHWIIH GRPEKDMHGYDIRRFHHSLTDHPRWIRERSHESYAKNYS VVFPHDEPLAGRNMRRDPLHEELLGQGCVFQERHGWERP GWFHPRGPAPVLEYDYYGAYGSRAHEDYAYRRLLADEYT FAFPPHHDTIKKECLACRGAAAVFDMSYFGKFYLVGLDA RKAADWLFSADVSRPPGSTVYTCMLNHRGGTESDLTVSR LAPSHQASPLAPAFEGDGYYLAMGGAVAQHNWSHITTVL QDQKSQCQLIDSSEDLGMISIQGPASRAILQEVLDADLS NEAFPFSTHKLLRAAGHLVRAMRLSFVGELGWELHIPKA SCVPVYRAVMAAGAKHGLINAGYRAIDSLSIEKGYRHWH ADLRPDDSPLEAGLAFTCKLKSPVPFLGREALEQQRAAG LRRRLVCFTMEDKVPMFGLEAIWRNGQVVGHVRRADFGF AIDKTIAYGYIHDPSGGPVSLDFVKSGDYALERMGVTYGAQAHLKSPFDPNNKRVKGIY |
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Database cross reference |
RefSeq Protein accession:NP_001128179
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Liver relevance
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HCC significant Proteins |
Yes/No |
Yes |
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Quality score |
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HLPP validation |
Yes/No |
Yes |
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Project name |
Chinese Liver;French Liver;Human Fetal Liver;Human Liver Organelles; |
Ontology annotation
| GO-C |
GO:0005759;C:mitochondrial matrix;IEA:UniProtKB-SubCell. |
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GO-F |
GO:0004047;F:aminomethyltransferase activity;IEA:InterPro. GO:0008480;F:sarcosine dehydrogenase activity;IEA:EC. |
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GO-P |
GO:0006546;P:glycine catabolic process;IEA:InterPro. GO:0055114;P:oxidation-reduction process;IEA:UniProtKB-KW. |
Post-translational modification
| LiverAtlas Protein ID |
MOD type1 |
Position1 |
Residue1 |
Source name1 |
source ID1 |
Source method |
HLPP validation1 (Yes/no) |
Quality score |
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HuLPr45229 |
PHOSPHORYLATION |
867 |
Y |
PhosphoSitePlus |
LTP |
N |
 
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Pathway
| Pathway name | |
| Pathway name |
