Anti-MECR Antibody Picoband®

SKU: A08189-1

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: WB,IHC,Flow Cytometry,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human, Mouse, Rat
Immunohistochemistry(Paraffin-embedded Section), 1-2 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: MECR

Specificity:

Background: Trans-2-enoyl-CoA reductase, mitochondrial is an enzyme that in humans is encoded by the MECR gene. The protein encoded by this gene is an oxidoreductase that catalyzes the last step in mitochondrial fatty acid synthesis. Defects in this gene are a cause of childhood-onset dystonia and optic atrophy.

Immunogen: E.coli-derived human MECR recombinant protein (Position: E39-A359).

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Heimer, G., Keratar, J. M., Riley, L. G., Balasubramaniam, S., Eyal, E., Pietikainen, L. P., Hiltunen, J. K., Marek-Yagel, D., Hamada, J., Gregory, A., Rogers, C., Hogarth, P., and 24 others. MECR mutations cause childhood-onset dystonia and optic atrophy, a mitochondrial fatty acid synthesis disorder. Am. J. Hum. Genet. 99: 1229-1244, 2016. 2. Liu, Z., Shimura, M., Zhang, L., Zhang, W., Wang, J., Ogawa-Tominaga, M., Wang, J., Wang, X., Lv, J., Shi, W., Zhang, V. W., Murayama, K., Fang, F. Whole exome sequencing identifies a novel homozygous MECR mutation in a Chinese patient with childhood-onset dystonia and basal ganglia abnormalities, without optic atrophy. Mitochondrion 57: 222-229, 2021. 3. Miinalainen, I. J., Chen, Z.-J., Torkko, J. M., Pirila, P. L., Sormunen, R. T., Bergmann, U., Qin, Y.-M., Hiltunen, J. K. Characterization of 2-enoyl thioester reductase from mammals: an ortholog of Ybr026p/Mrf1'p of the yeast mitochondrial fatty acid synthesis type II. J. Biol. Chem. 278: 20154-20161, 2003.

Uniprot ID: Q9BV79

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 38 kDa

Calculated Molecular Weight:

Gene ID: 51102

Protein Name: Enoyl-[acyl-carrier-protein] reductase, mitochondrial

Gene Full Name: mitochondrial trans-2-enoyl-CoA reductase

Synonyms: CGI 63; CGI-63; FASN2B; HsNrbf 1; MECR; NBRF1; NRBF 1; NRBF1; HsNrbf-1; NRBF-1