Mouse anti-Human NDUFS4 Monoclonal Antibody | anti-NDUFS4 antibody

NDUFS4 (NADH Dehydrogenase (Ubiquinone) Fe-S Protein 4, Mitochondrial, NADH-Ubiquinone Oxidoreductase 18kD Subunit, Complex I-18kD, CI-18kD, Complex I-AQDQ, CI-AQDQ) (AP)

Cat. Num. AAA6132524

• Gene Names: NDUFS4; AQDQ; CI-18; CI-AQDQ; CI-18 kDa
• Reactivity: Human
• Applications: ELISA, Immunohistochemistry, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: NDUFS4; Monoclonal Antibody; NDUFS4 (NADH Dehydrogenase (Ubiquinone) Fe-S Protein 4; Mitochondrial; NADH-Ubiquinone Oxidoreductase 18kD Subunit; Complex I-18kD; CI-18kD; Complex I-AQDQ; CI-AQDQ) (AP); anti-NDUFS4 antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG2a,k
• Clone Number: 1A1
• Specificity: Recognizes human NDUFS4.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Alkaline Phosphatase (AP).

• Applicable Applications for anti-NDUFS4 antibody: ELISA (EIA), Immunohistochemistry (IHC) Paraffin, Western Blot (WB)
• Application Notes: IHC-P: 1ug/ml; Applications are based on unconjugated antibody.
• Immunogen: Partial recombinant corresponding to aa66-175 from human NDUFS4 (NP_002486) with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: GVPEEHIKTRKVRIFVPARNNMQSGVNNTKKWKMEFDTRERWENPLMGWASTADPLSNMVLTFSTKEDAVSFAEKNGWSYDIEERKVPKPKSKSYGANFSWNKRTRVSTK
• Conjugate: AP
• Preparation and Storage: Store product at 4 degree C. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western Blot detection against Immunogen (37.84kD).)

Western Blot (WB)

(NDUFS4 monoclonal antibody Western Blot analysis of NDUFS4 expression in A-431.)

Western Blot (WB)

(Western Blot analysis of NDUFS4 expression in transfected 293T cell line by NDUFS4 monoclonal antibody. Lane 1: NDUFS4 transfected lysate (20.1kD). Lane 2: Non-transfected lysate.)

Immunohistochemistry (IHC)

(Immunoperoxidase of monoclonal antibody to NDUFS4 on formalin-fixed paraffin-embedded human colon. [antibody concentration 1ug/ml])

Testing Data

(Detection limit for recombinant GST tagged NDUFS4 is ~0.3ng/ml as a capture antibody.)

Western Blot (WB)

(Western blot analysis of NDUFS4 over-expressed 293 cell line, cotransfected with NDUFS4 Validated Chimera RNAi (Lane 2) or non-transfected control (Lane 1). Blot probed with NDUFS4 monoclonal antibody. GAPDH (36.1kD) used as specificity and loading control.)

Product Categories/Family for anti-NDUFS4 antibody

Antibodies; Enzymes; Dehydrogenase

NCBI and Uniprot Product Information

• NCBI GI #: 4505369
• NCBI GeneID: 4724
• NCBI Accession #: NP_002486
• NCBI GenBank Nucleotide #: NM_002495
• UniProt Accession #: O43181; Q9BS69
• Molecular Weight: 15.5 kDa (134aa), confirmed by MALDI-TOF.
• NCBI Official Full Name: NADH dehydrogenase
• NCBI Official Synonym Full Names: NADH:ubiquinone oxidoreductase subunit S4
• NCBI Official Symbol: NDUFS4
• NCBI Official Synonym Symbols: AQDQ; CI-18; CI-AQDQ; CI-18 kDa
• NCBI Protein Information: NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial
• UniProt Protein Name: NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial
• Protein Family: NADH dehydrogenase [ubiquinone] iron-sulfur protein
• UniProt Gene Name: NDUFS4
• UniProt Synonym Gene Names: CI-18 kDa; CI-AQDQ
• UniProt Entry Name: NDUS4_HUMAN

NCBI Description

This gene encodes an nuclear-encoded accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I, or NADH:ubiquinone oxidoreductase). Complex I removes electrons from NADH and passes them to the electron acceptor ubiquinone. Mutations in this gene can cause mitochondrial complex I deficiencies such as Leigh syndrome. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2015]

Uniprot Description

NDUFS4: Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. Defects in NDUFS4 are a cause of mitochondrial complex I deficiency (MT-C1D). A disorder of the mitochondrial respiratory chain that causes a wide range of clinical disorders, from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease. Belongs to the complex I NDUFS4 subunit family.

Protein type: EC 1.6.99.3; Oxidoreductase; Energy Metabolism - oxidative phosphorylation; Mitochondrial; EC 1.6.5.3

Chromosomal Location of Human Ortholog: 5q11.1

Cellular Component: mitochondrion; mitochondrial inner membrane; mitochondrial respiratory chain complex I

Molecular Function: NADH dehydrogenase (ubiquinone) activity

Biological Process: cellular metabolic process; mitochondrial respiratory chain complex I assembly; positive regulation of fibroblast proliferation; cAMP-mediated signaling; regulation of protein amino acid phosphorylation; response to cAMP; mitochondrial electron transport, NADH to ubiquinone; cellular respiration; brain development

Disease: Leigh Syndrome; Mitochondrial Complex I Deficiency