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SDHA (human)

Overview SDHA is the catalytic subunit of succinate dehydrogenase (SDH) complex II of the mitochondrial electron transport chain. Complex II contains four subunits: the flavoprotein catalytic subunit SDHA, iron-sulfur protein SDHB, and a cytochrome b560 composed of SDHC and SDHD. Interaction with SDH5 is required for FAD attachment. Responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). Defects in SDHA cause defective mitochondrial oxidative phosphorylation, giving rise to heterogeneous clinical symptoms ranging from isolated organ dysfunction to multisystem disorder. Acetylation of SDHA may control entry of the substrate into the active site, thus regulating its enzymatic activity. Acetylated SDHA may be a SIRT3 substrate SIRT3 is a mitochondrial NAD(+)-dependent deacetylase. Increased succinate levels as a consequence of SDH deficiency inhibit hypoxia inducible factor-1alpha (HIF-1alpha) prolyl hydroxylases leading to sustained HIF-1alpha expression in tumours. Defects in SDHA cause of Leigh syndrome, a severe disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions. Note: This description may include information from UniProtKB. Protein type: Carbohydrate Metabolism - citrate (TCA) cycle; EC 1.3.5.1; Oxidoreductase; Energy Metabolism - oxidative phosphorylation; Mitochondrial Cellular Component: mitochondrial respiratory chain complex II; mitochondrion; mitochondrial inner membrane Molecular Function: succinate dehydrogenase (ubiquinone) activity; protein binding; FAD binding; succinate dehydrogenase activity Biological Process: nervous system development; succinate metabolic process; tricarboxylic acid cycle Reference #:  P31040 (UniProtKB) Alt. Names/Synonyms: DHSA; Flavoprotein subunit of complex II; Fp; SDH1; SDH2; SDHA; SDHF; Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial; succinate dehydrogenase complex flavoprotein subunit; succinate dehydrogenase complex, subunit A, flavoprotein (Fp) Gene Symbols: SDHA Molecular weight: 72,692 Da Basal Isoelectric point: 7.06  Predict pI for various phosphorylation states Protein-Specific Antibodies or siRNAs from Cell Signaling Technology®

Select Structure to View Below SDHA

STRING  |  Scansite  |  Phospho.ELM  |  NetworKIN  |  Pfam  |  ENZYME  |  DISEASE 252011 256000 600857  |  Source  |  InnateDB  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene

	Modification Sites and Domains	Show Modification Legend
Click here to view phosphorylation modifications only

	Modification Sites in Parent Protein, Orthologs, and Isoforms	Show Modification Legend

SS  SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.	MS  MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.			human
0	1		T24-p	ALAKAWPtVLQTGTR
0	1		K92-u	FNTACVTkLFPTRSH
1	10		K179-a	AFGGQSLkFGkGGQA
0	3		K179-u	AFGGQSLkFGkGGQA
0	8		K182-a	GQSLkFGkGGQAHRC
1	0		Y215-p	SLRYDTSyFVEYFAL
0	1		K250	SIHRIRAKNTVVATG
0	1		K250-u	SIHRIRAkNTVVATG
0	3		K335-a	ERYAPVAkDLASRDV
0	2		K335-u	ERYAPVAkDLASRDV
0	1		Y365-p	GPEKDHVyLQLHHLP
0	1		R423	NYKGQVLRHVNGQDQ
0	1		R423	NYKGQVLRHVNGQDQ
0	2		S456-p	ANRLGANsLLDLVVF
0	1		K480	ESCRPGDKVPPIKPN
1	3		K485	GDKVPPIKPNAGEES
1	13		K498-a	ESVMNLDkLRFADGS
0	1		K498	ESVMNLDKLRFADGS
0	1		S509	ADGSIRTSELRLSMQ
0	5		K517-a	ELRLSMQkSMQNHAA
0	2		K517-u	ELRLSMQkSMQNHAA
0	1		S530	AAVFRVGSVLQEGCG
1	12		K538	VLQEGCGKISkLYGD
0	2		K541-a	EGCGKISkLYGDLkH
2	0		Y543	CGKISkLYGDLkHLk
0	17		K547-a	SkLYGDLkHLkTFDR
0	1		K547	SkLYGDLKHLkTFDR
0	6		K550-a	YGDLkHLkTFDRGMV
0	20		K598-a	AHAREDYkVRIDEyD
2	2		Y604-p	YkVRIDEyDySkPIQ
0	1		Y606-p	VRIDEyDySkPIQGQ
0	16		K608-a	IDEyDySkPIQGQQK
0	2		K608-u	IDEyDySkPIQGQQK
0	1		K624	PFEEHWRKHTLSyVD
0	19		Y629-p	WRKHTLSyVDVGTGK
0	1		G633	TLSyVDVGTGKVTLE
0	1		G633	TLSyVDVGTGKVTLE
0	1		T638	DVGTGKVTLEYRPVI
0	1		Y641	TGKVTLEYRPVIDKT

	mouse
G24	ALTGAWPGTLQKQTC
K92	FNTACLTKLFPTRSH
K179-a	AFGGQSLkFGkGGQA
K179-u	AFGGQSLkFGkGGQA
K182-a	GQSLkFGkGGQAHRC
Y215	SLRYDTSYFVEYFAL
K250-a	SIHRIRAkNTVIATG
K250	SIHRIRAKNTVIATG
K335-a	ERYAPVAkDLASRDV
K335-u	ERYAPVAkDLASRDV
Y365	GPEKDHVYLQLHHLP
K423-a	NYKGQVLkHVNGQDQ
K423-m1	NYKGQVLkHVNGQDQ
S456	ANRLGANSLLDLVVF
K480-a	ESCRPGDkVPSIkAN
K485-a	GDkVPSIkANAGEES
K498-a	ESVMNLDkLRFADGS
K498-u	ESVMNLDkLRFADGS
S509-p	ADGSIRTsELRLNMQ
K517-a	ELRLNMQkSMQNHAA
K517-u	ELRLNMQkSMQNHAA
S530-p	AAVFRVGsVLQEGCE
K538-a	VLQEGCEkISQLYGD
Q541	EGCEkISQLYGDLkH
Y543	CEkISQLYGDLkHLk
K547-a	SQLYGDLkHLkTFDR
K547-u	SQLYGDLkHLkTFDR
K550-a	YGDLkHLkTFDRGMV
K598-a	AHAREDYkVRVDEYD
Y604	YkVRVDEYDYSkPIQ
Y606	VRVDEYDYSkPIQGQ
K608-a	VDEYDYSkPIQGQQK
K608	VDEYDYSKPIQGQQK
K624-a	PFGEHWRkHTLSyVD
Y629-p	WRkHTLSyVDIkTGK
K633-a	TLSyVDIkTGKVtLE
K633-u	TLSyVDIkTGKVtLE
T638-p	DIkTGKVtLEyRPVI
Y641-p	TGKVtLEyRPVIDKT

	rat
-	gap
K84	FNTACLTKLFPTRSH
K171	AFGGQSLKFGKGGQA
K171	AFGGQSLKFGKGGQA
K174	GQSLKFGKGGQAHRC
Y207	SLRYDTSYFVEYFAL
K242	SIHRIRAKNTIIATG
K242	SIHRIRAKNTIIATG
K327	ERYAPVAKDLASRDV
K327	ERYAPVAKDLASRDV
Y357	GPEKDHVYLQLHHLP
K415	NYKGQVLKHVNGQDQ
K415	NYKGQVLKHVNGQDQ
S448	ANRLGANSLLDLVVF
K472	ESCRPGDKVPPIKAN
K477	GDKVPPIKANAGEES
K490	ESVMNLDKLRFADGS
K490	ESVMNLDKLRFADGS
S501	ADGSVRTSELRLSMQ
K509	ELRLSMQKSMQSHAA
K509	ELRLSMQKSMQSHAA
S522	AAVFRVGSVLQEGCE
K530	VLQEGCEKVSQLyGD
Q533	EGCEKVSQLyGDLQH
Y535-p	CEKVSQLyGDLQHLk
Q539	SQLyGDLQHLkTFDR
Q539	SQLyGDLQHLkTFDR
K542-a	yGDLQHLkTFDRGMV
K590	AHAREDYKVRIDEyD
Y596-p	YKVRIDEyDYSKPIE
Y598	VRIDEyDYSKPIEGQ
K600	IDEyDYSKPIEGQQK
K600	IDEyDYSKPIEGQQK
K616	PFAEHWRKHTLSYVD
Y621	WRKHTLSYVDTKTGK
K625	TLSYVDTKTGKVTLD
K625	TLSYVDTKTGKVTLD
T630	DTKTGKVTLDYRPVI
Y633	TGKVTLDYRPVIDKT

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