Rabbit Anti-TMLHE Polyclonal Antibody – Cat# MBS6356562

TMLHE Antibody (N-term) western blot analysis in HepG2 cell line lysates (35ug/lane).This demonstrates the TMLHE antibody detected the TMLHE protein (arrow).

TMLHE Antibody – Rabbit Polyclonal

Name: TMLHE Antibody; Polyclonal; Rabbit; Human-Reactive; MyBioSource
Brand: MyBioSource
SKU: MBS6356562
Price: 1060 USD
Availability: InStock
Rating: 5 (10 reviews)

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS6356562

This gene encodes the protein trimethyllysine dioxygenase which is the first enzyme in the carnitine biosynthesis pathway. Carnitine play an essential role in the transport of activated fatty acids across the inner mitochondrial membrane. The encoded protein… More details

$1060 / 0.2 mL +1 FREE 8GB USB

Product Documentation

Sample Manual Insert View PDF

Testing Data View PDF

Certificate of Analysis (COA) View PDF

QC Report View PDF

Material Safety Data Sheet View PDF

Technical Datasheet View PDF

Protocol View PDF

Product Overview

Online Datasheet in PDF

Product Name

TMLHE, Polyclonal Antibody

Full Product Name

TMLHE, NT (TMLHE, TMLH, Trimethyllysine dioxygenase, mitochondrial, Epsilon-trimethyllysine 2-oxoglutarate dioxygenase, Epsilon-trimethyllysine hydroxylase, TML hydroxylase, TML-alpha-ketoglutarate dioxygenase) (FITC)

Product Gene Name

anti-TMLHE antibody

Product Synonym Names

TMLHE; TMLH; Trimethyllysine dioxygenase, mitochondrial; Epsilon-trimethyllysine 2-oxoglutarate dioxygenase; Epsilon-trimethyllysine hydroxylase; TML hydroxylase; TML-alpha-ketoglutarate dioxygenase

Clonality

Polyclonal

Isotype

IgG

Host

Rabbit

Species Reactivity

Human

Form/Format

Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).

Purity/Purification

Purified by Protein A Affinity Chromatography.

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. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Molecular Weight

50,832 Da

OMIM

gene 300872

3D Structure

ModBase 3D Structure for Q9NVH6

Immunogen

TMLHE antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 123-153 amino acids from the N-terminal region of human TMLHE.

Conjugate

FITC

Note

Preservative Free

Special Handling

Light sensitive

Product Categories/Family for anti-TMLHE antibody

Antibodies; Enzymes; Oxidase

Applications Tested/Suitable for anti-TMLHE antibody

Western Blot (WB), FLISA

Application Notes for anti-TMLHE antibody

WB: 1:100-500
FLISA: 1:1,000
Applications are based on unconjugated antibody.

Product References and Citations for anti-TMLHE antibody

Monfregola, J., et al. Gene 395 (1-2), 86-97 (2007): Monfregola, J., et al. J. Cell. Physiol. 204(3):839-847(2005). Vaz, F.M., et al. J. Biol. Chem. 276(36):33512-33517(2001). Rogner, U.C., et al. Genome Res. 6(10):922-934(1996).

Other Notes

Small volumes of anti-TMLHE antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Searchable Terms for TMLHE purchase

MBS6356562 is an antibody targeting TMLHE. Also known as TMLHE, NT (TMLHE, TMLH, Trimethyllysine dioxygenase, mitochondrial, Epsilon-trimethyllysine 2-oxoglutarate dioxygenase, Epsilon-trimethyllysine hydroxylase, TML hydroxylase, TML-alpha-ketoglutarate dioxygenase) (FITC). Reactive in Human. Host: Rabbit.

Additional Details

NCBI/Uniprot data below describe general gene information for TMLHE. It may not necessarily be applicable to this product.

NCBI GI #

8922625

NCBI GeneID

55217

NCBI Accession #

NP_060666.1

UniProt Primary Accession #

Q9NVH6

UniProt Secondary Accession #

Q5TZB5; Q6IA90; Q8TBT0; A8K6M9; B4E3R3

UniProt Related Accession #

Q9NVH6

GenBank Nucleotide #

NM_018196.3

NCBI Official Full Name

trimethyllysine dioxygenase, mitochondrial isoform 1

NCBI Official Synonym Full Names

trimethyllysine hydroxylase, epsilon

NCBI Official Symbol

TMLHE

NCBI Official Synonym Symbols

TMLD; TMLH; BBOX2; AUTSX6; TMLHED; XAP130

UniProt Protein Name

Trimethyllysine dioxygenase, mitochondrial

UniProt Synonym Protein Names

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

UniProt Gene Name

TMLHE

UniProt Synonym Gene Names

TMLH; TML dioxygenase; TMLD

UniProt Entry Name

TMLH_HUMAN

NCBI Protein Information

trimethyllysine dioxygenase, mitochondrial

UniProt Comments for TMLHE

TMLHE: Converts trimethyllysine (TML) into hydroxytrimethyllysine (HTML). Defects in TMLHE are the cause of epsilon-trimethyllysine hydroxylase deficiency (TMLHED). An inborn error of carnitine biosynthesis associated with an increased risk for developing autistic behavior. Autism is a complex multifactorial, pervasive developmental disorder characterized by impairments in reciprocal social interaction and communication, restricted and stereotyped patterns of interests and activities, and the presence of developmental abnormalities by 3 years of age. Most individuals with autism also manifest moderate mental retardation. Belongs to the gamma-BBH/TMLD family. 8 isoforms of the human protein are produced by alternative promoter.

Protein type: Mitochondrial; Oxidoreductase; Amino Acid Metabolism - lysine degradation; EC 1.14.11.8

Chromosomal Location of Human Ortholog: Xq28

Cellular Component: mitochondrion; mitochondrial matrix

Molecular Function: oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen; trimethyllysine dioxygenase activity; L-ascorbic acid binding; iron ion binding

Biological Process: negative regulation of oxidoreductase activity; carnitine biosynthetic process

Disease: Epsilon-trimethyllysine Hydroxylase Deficiency

Research Articles on TMLHE

1. Case Report: complex recombination with deletion in the F8 and duplication in the TMLHE mediated by int22h copies during early embryogenesis in proband's mother.

Precautions

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

Disclaimer

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.

Product Note

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual, it is intended to serve as an example only. Please refer to the instructions For Use provided with the assay kit for precise details.

Product PubMed Publications

Products associated with anti-TMLHE antibody

Reference Product	PubMed Publications
ALDH9A1 antibody	>2 publications with TMLHE and ALDH9A1
VBP1 antibody	>1 publications with TMLHE and VBP1
RAB39B antibody	>1 publications with TMLHE and RAB39B
FUNDC2 antibody	>1 publications with TMLHE and FUNDC2

Pathways associated with anti-TMLHE antibody

Products by Pathway	Pathway Diagram
Carnitine Synthesis Pathway antibodies	Carnitine Synthesis Pathway Diagram
L-carnitine Biosynthesis Pathway antibodies	L-carnitine Biosynthesis Pathway Diagram
L-carnitine Biosynthesis Pathway antibodies	L-carnitine Biosynthesis Pathway Diagram
Lysine Degradation Pathway antibodies	Lysine Degradation Pathway Diagram
Lysine Degradation Pathway antibodies	Lysine Degradation Pathway Diagram
Metabolism Pathway antibodies	Metabolism Pathway Diagram
Metabolism Of Amino Acids And Derivatives Pathway antibodies	Metabolism Of Amino Acids And Derivatives Pathway Diagram

Diseases associated with anti-TMLHE antibody

Disease Name	Pubmed Publications
Mental Disorders Antibodies	>3 publications with TMLHE and Mental Disorders
Fatty Liver Antibodies	>2 publications with TMLHE and Fatty Liver
Blood Coagulation Disorders Antibodies	>1 publications with TMLHE and Blood Coagulation Disorders

Organs/Tissues associated with anti-TMLHE antibody

Organ/Tissue Name	Pubmed Publications
Blood Antibodies	>3 publications with TMLHE and Blood
Liver Antibodies	>2 publications with TMLHE and Liver