Alternative Name(s):

Glomerulosclerosis; Mpv17; Mpv17 human homolog of glomerulosclerosis and nephrotic syndrome; MpV17 mitochondrial inner membrane protein; MPV17_HUMAN; MTDPS6; Protein Mpv17; SYM1

Species: (Organism)

Homo sapiens (Human)

Gene Names:

MPV17

Tag info:

N-terminal 6xHis-SUMO-tagged

Target Protein AA Sequence:

MALWRAYQRALAAHPWKVQVLTAGSLMGLGDIISQQLVERRGLQEHQRGRTLTMVSLGCGFVGPVVGGWYKVLDRFIPGTTKVDALKKMLLDQGGFAPCFLGCFLPLVGALNGLSAQDNWAKLQRDYPDALITNYYLWPAVQLANFYLVPLHYRLAVVQCVAVIWNSYLSWKAHRL

Expression Region:

1-176aa

Subcellular Location:

Mitochondrion inner membrane, Multi-pass membrane protein

Tissue Specificity:

Ubiquitous. Expressed in pancreas, kidney, muscle, liver, lung, placenta, brain and heart.

Protein Length:

Full Length

Pathway:

Mol. Weight:

35.7 kDa

Purity:

Greater than 90% as determined by SDS-PAGE.

Form:

Liquid or Lyophilized powder

Buffer:

If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.

Research Areas:

Metabolism

Function:

Involved in mitochondria homeostasis. May be involved in the metabolism of reactive oxygen species and control of oxidative phosphorylation and mitochondrial DNA (mtDNA) maintenance.

Involvement in disease:

Mitochondrial DNA depletion syndrome 6 (MTDPS6)

Relevance:

Involved in mitochondria homeostasis. May be involved in the metabolism of reactive oxygen species and control of oxidative phosphorylation and mitochondrial DNA (mtDNA) maintenance.

Reconstitution:

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.

Protein Families:

Peroxisomal membrane protein PXMP2/4 family

Reference:

Identification of rare DNA variants in mitochondrial disorders with improved array-based sequencing.Wang W., Shen P., Thiyagarajan S., Lin S., Palm C., Horvath R., Klopstock T., Cutler D., Pique L., Schrijver I., Davis R.W., Mindrinos M., Speed T.P., Scharfe C.Nucleic Acids Res. 39:44-58(2011)