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

NIFUN (Iron-Sulfur Cluster Assembly Enzyme ISCU, Mitochondrial, NifU-like N-Terminal Domain-Containing Protein, NifU-like Protein, ISCU) (Biotin)

Cat. Num. AAA6143184

• Gene Names: ISCU; HML; ISU2; NIFU; NIFUN; hnifU; 2310020H20Rik
• Reactivity: Human
• Applications: ELISA, Immunofluorescence, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: NIFUN; Monoclonal Antibody; NIFUN (Iron-Sulfur Cluster Assembly Enzyme ISCU; Mitochondrial; NifU-like N-Terminal Domain-Containing Protein; NifU-like Protein; ISCU) (Biotin); anti-NIFUN antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG1,k
• Clone Number: 3B8-1C4
• Specificity: Recognizes human NIFUN.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.

• Applicable Applications for anti-NIFUN antibody: ELISA (EIA), Immunofluorescence (IF), Western Blot (WB)
• Application Notes: IF: 10ug/ml; Applications are based on unconjugated antibody.
• Immunogen: Full length recombinant corresponding to aa26-167 from NIFUN (AAH11906) with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: RELSAPARLYHKKVVDHYENPRNVGSLDKTSKNVGTGLVGAPACGDVMKLQIQVDEKGKIVDARFKTFGCGSAIASSSLATEWVKGKTVEEALTIKNTDIAKELCLPPVKLHCSMLAEDAIKAALADYKLKQEPKKGEAEKK*
• Conjugate: Biotin
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. 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 after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot detection against Immunogen (41.73kD).)

Western Blot (WB)

(NIFUN monoclonal antibody Western Blot analysis of NIFUN expression in HL-60.)

Immunofluorescence (IF)

(Immunofluorescence of monoclonal antibody to NIFUN on HeLa cell. [antibody concentration 10ug/ml])

Testing Data

(Detection limit for recombinant GST tagged NIFUN is ~0.03ng/ml as a capture antibody.)

Product Categories/Family for anti-NIFUN antibody

Antibodies; Mitochondrial Proteins

NCBI and Uniprot Product Information

• NCBI GI #: 33989202
• NCBI GeneID: 23479
• UniProt Accession #: Q9H1K1; Q6P713; Q99617; Q9H1K2
• Molecular Weight: 17,999 Da
• NCBI Official Full Name: Homo sapiens iron-sulfur cluster scaffold homolog (E. coli), mRNA
• NCBI Official Synonym Full Names: iron-sulfur cluster scaffold homolog (E. coli)
• NCBI Official Symbol: ISCU
• NCBI Official Synonym Symbols: HML; ISU2; NIFU; NIFUN; hnifU; 2310020H20Rik
• NCBI Protein Information: iron-sulfur cluster assembly enzyme ISCU, mitochondrial; nifU-like protein; NifU-like N-terminal domain containing; IscU iron-sulfur cluster scaffold homolog; nifU-like N-terminal domain-containing protein
• UniProt Protein Name: Iron-sulfur cluster assembly enzyme ISCU, mitochondrial
• UniProt Gene Name: ISCU
• UniProt Synonym Gene Names: NIFUN
• UniProt Entry Name: ISCU_HUMAN

NCBI Description

Iron-sulfur (Fe-S) clusters are necessary for several mitochondrial enzymes and other subcellular compartment proteins. They contain sulfur and iron, and are created via several steps that include cysteine desulfurases, iron donors, chaperones, and scaffold proteins. This gene encodes the two isomeric forms, ISCU1 and ISCU2, of the Fe-S cluster scaffold protein. Mutations in this gene have been found in patients with myopathy with severe exercise intolerance and myoglobinuria. [provided by RefSeq, Jul 2008]

Uniprot Description

ISCU: Involved in the assembly or repair of the [Fe-S] clusters present in iron-sulfur proteins. Binds iron. Defects in ISCU are the cause of myopathy with exercise intolerance Swedish type (MEIS); also known as myopathy with deficiency of succinate dehydrogenase and aconitase or myoglobinuria due to abnormal glycolysis or hereditary myopathy with lactic acidosis (HML). This autosomal recessive metabolic disease is characterized by lifelong severe exercise intolerance, in which minor exertion causes fatigue of active muscles, shortness of breath, and cardiac palpitations in association with lactic acidosis. The biochemical phenotype is characterized by a deficiency in mitochondrial iron-sulfur proteins and impaired muscle oxidative metabolism. Belongs to the NifU family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial

Chromosomal Location of Human Ortholog: 12q24.1

Cellular Component: mitochondrion; mitochondrial matrix; cytoplasm; cytosol; nucleus

Molecular Function: protein binding; iron ion binding; iron-sulfur cluster binding; protein complex scaffold

Biological Process: iron-sulfur cluster assembly; nitrogen fixation

Disease: Myopathy With Lactic Acidosis, Hereditary