Alternative Name(s):

Epsilon-trimethyllysine 2-oxoglutarate dioxygenase;Epsilon-trimethyllysine hydroxylaseTML hydroxylaseTML-alpha-ketoglutarate dioxygenase ;TML dioxygenase ;TMLD

Species: (Organism)

Homo sapiens (Human)

Gene Names:

TMLHE

Tag info:

N-terminal 6xHis-tagged

Target Protein AA Sequence:

LLKGGVIYPALPQPNFKSLLPLAVHWHHTASKSLTCAWQQHEDHFELKYANTVMRFDYVWLRDHCRSASCYNSKTHQRSLDTASVDLCIKPKTIRLDETTLFFTWPDGHVTKYDLNWLVKNSYEGQKQKVIQPRILWNAEIYQQAQVPSVDCQSFLETNEGLKKFLQNFLLYGIAFVENVPPTQEHTEKLAERISLIRETIYGRMWYFTSDFSRGDTAYTKLALDRHTDTTYFQEPCGIQVFHCLKHEGTGGRTLLVDGFYAAEQVLQKAPEEFELLSKVPLKHEYIEDVGECHNHMIGIGPVLNIYPWNKELYLIRLFKEKQNTVNRQWNSSLQCDIPERILTYRHFVSGTSIEHRGSLI

Expression Region:

16-376aa

Subcellular Location:

Mitochondrion matrix

Tissue Specificity:

All isoforms, but isoform 8, are widely expressed in adult and fetal tissues. Isoform 8 is restricted to heart and skeletal muscle.

Protein Length:

Full Length of Isoform 4

Pathway:

Mol. Weight:

46.1 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:

Converts trimethyllysine (TML) into hydroxytrimethyllysine (HTML)

Involvement in disease:

Autism, X-linked 6 (AUTSX6)

Relevance:

Converts trimethyllysine (TML) into hydroxytrimethyllysine (HTML).

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:

Gamma-BBH/TMLD family

Reference:

Molecular and biochemical characterization of rat epsilon-N-trimethyllysine hydroxylase, the first enzyme of carnitine biosynthesis.Vaz F.M., Ofman R., Westinga K., Back J.W., Wanders R.J.A.J. Biol. Chem. 276:33512-33517(2001)