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The antibody against TMLHE was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 167-376 of human TMLHE (NP_001171726.1) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on IF/ICC, ELISA.
The antibody against TMLHE was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 167-376 of human TMLHE (NP_001171726.1) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on IF/ICC, ELISA.
| Cat.No | ADA-02577A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | TMLHE |
| Target Synonyms | TMLD; TMLH; BBOX2; AUTSX6; TMLHED; XAP130; TMLHE | Form | Liquid |
| Species Reactivity | Human | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.02% sodium azide, pH7.3. | Purification Method | Affinity purification |
| Application | ELISA, IF/ICC |
| Immunogen Description | Recombinant fusion protein containing a sequence corresponding to amino acids 167-376 of human TMLHE (NP_001171726.1). | Target Species | Human |
|---|---|---|---|
| Uniprot ID | Q9NVH6 | Immunogen Sequence |
Uniprot Id
Q9NVH6
Target Species
Human
Target Name
TMLHE
Target Full Name
Trimethyllysine dioxygenase, mitochondrial
Target Function
Converts trimethyllysine (TML) into hydroxytrimethyllysine (HTML).
Target Involvement
Autism, X-linked 6 (AUTSX6)
Target Subcellular Location
Mitochondrion matrix.
Target Protein Families
Gamma-BBH/TMLD family
Target Tissue Specificity
All isoforms, but isoform 8, are widely expressed in adult and fetal tissues. Isoform 8 is restricted to heart and skeletal muscle.
Target Research Area
Metabolism
Target Synonyms
AUTSX6; BBOX2; Butyrobetaine (gamma) 2 oxoglutarate dioxygenase (gamma butyrobetaine hydroxylase) 2; Epsilon-trimethyllysine 2-oxoglutarate dioxygenase; Epsilon-trimethyllysine hydroxylase; FLJ10727; TML dioxygenase; TML hydroxylase; TML-alpha-ketoglutarate dioxygenase; TMLD; TMLH; TMLH_HUMAN; TMLHE; TMLHED; Trimethyllysine dioxygenase, mitochondrial; Trimethyllysine hydroxylase epsilon; XAP130
Target Background
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 converts trimethyllysine into hydroxytrimethyllysine. A pseudogene of this gene is found on chromosome X. Alternate splicing results in multiple transcript variants.
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