Recombinant Human NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 (NDUFA13)
Shipped with Ice Packs 
In Stock 
Description
Introduction to Recombinant Human NADH Dehydrogenase [Ubiquinone] 1 Alpha Subcomplex Subunit 13 (NDUFA13)
Recombinant Human NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 (NDUFA13) is a protein subunit encoded by the NDUFA13 gene, located on chromosome 19. This protein is an accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase, also known as Complex I, which plays a crucial role in the electron transport chain. NDUFA13 is involved in transferring electrons from NADH to the respiratory chain, with ubiquinone serving as the immediate electron acceptor .
Structure and Function of NDUFA13
NDUFA13 encodes a 17 kDa protein with 144 amino acids. The protein structure includes a long hydrophobic transmembrane domain and a hydrophilic domain. It is primarily composed of alpha helices, with its carboxy-terminal half potentially forming a coiled-coil structure. The amino-terminal part contains a putative beta sheet rich in hydrophobic amino acids, which may act as a mitochondrial import signal .
| Characteristics | Description |
|---|---|
| Molecular Weight | Approximately 17 kDa |
| Amino Acids | 144 amino acids |
| Structure | Hydrophobic transmembrane domain and hydrophilic domain |
| Secondary Structure | Primarily alpha helices with potential coiled-coil in the carboxy-terminal half |
Role in Mitochondrial Function and Disease
NDUFA13 is essential for Complex I assembly and electron transfer activity. It binds to the signal transducers and activators of transcription 3 (STAT3) transcription factor and can function as a tumor suppressor . Diseases associated with NDUFA13 include Mitochondrial Complex I Deficiency and Thyroid Carcinoma, Hurthle Cell .
Research Findings on NDUFA13
Recent studies have highlighted the role of NDUFA13 in generating reactive oxygen species (ROS) through electron leak within Complex I. This ROS generation can serve as a second messenger, activating antiapoptotic signaling pathways. For instance, moderate down-regulation of NDUFA13 in cardiac-specific knockout mice resulted in increased cytoplasmic HO levels, which led to STAT3 dimerization and activation of antiapoptotic signaling. This protected the heart from ischemia-reperfusion injury by suppressing apoptosis .
| Experimental Model | Outcome |
|---|---|
| Cardiac-Specific NDUFA13 Heterozygous Knockout Mice | Increased cytoplasmic HO levels, STAT3 activation, and reduced infarct size during ischemia-reperfusion injury |
| H9C2 Cells with NDUFA13 siRNA | Moderate down-regulation of NDUFA13 conferred protection against hypoxia/reoxygenation-induced apoptosis |
References: Learn.MapMyGenome. NDUFA13 Gene: Function, Mutations, and Diseases. PNAS. Electron leak from NDUFA13 within mitochondrial complex I.... PMC. Synthesis and Structure–Activity Relationship Studies of N-Benzyl-2-phenylpyrimidin-4-amine Derivatives.... GeneCards. NDUFA13 Gene - NADH:Ubiquinone Oxidoreductase Subunit A13. MyGenome LOVD. Full data view for gene NDUFA13. PMC. Role of NADH Dehydrogenase (Ubiquinone) 1 alpha subcomplex 4-like 2 in clear cell renal cell carcinoma.
Product Specs
Form
Lyophilized powder
Note: While we prioritize shipping the format currently in stock, please specify your format preference in order notes for customized preparation.
Note: While we prioritize shipping the format currently in stock, please specify your format preference in order notes for customized preparation.
Lead Time
Delivery times vary depending on the purchase method and location. Contact your local distributor for precise delivery estimates.
Note: All proteins are shipped with standard blue ice packs unless dry ice shipping is requested in advance. Additional fees apply for dry ice shipping.
Note: All proteins are shipped with standard blue ice packs unless dry ice shipping is requested in advance. Additional fees apply for dry ice shipping.
Notes
Avoid repeated freeze-thaw cycles. Store working aliquots at 4°C for up to one week.
Reconstitution
Centrifuge the vial briefly before opening to consolidate the contents. Reconstitute the protein in sterile, deionized water to a concentration of 0.1-1.0 mg/mL. For long-term storage, we recommend adding 5-50% glycerol (final concentration) and aliquoting at -20°C/-80°C. Our standard glycerol concentration is 50% and serves as a guideline.
Shelf Life
Shelf life depends on storage conditions, buffer composition, temperature, and protein stability. Generally, liquid formulations have a 6-month shelf life at -20°C/-80°C, while lyophilized forms have a 12-month shelf life at -20°C/-80°C.
Storage Condition
Upon receipt, store at -20°C/-80°C. Aliquot for multiple uses to prevent repeated freeze-thaw cycles.
Tag Info
The tag type is determined during manufacturing.
Note: The tag type is determined during production. Please specify your required tag type for preferential development.
Note: The tag type is determined during production. Please specify your required tag type for preferential development.
Synonyms
NDUFA13; GRIM19; CDA016; CGI-39; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13; Cell death regulatory protein GRIM-19; Complex I-B16.6; CI-B16.6; Gene associated with retinoic and interferon-induced mortality 19 protein; GRIM-19; Gene associated with retinoic and IFN-induced mortality 19 protein; NADH-ubiquinone oxidoreductase B16.6 subunit
Buffer Before Lyophilization
Tris/PBS-based buffer, 6%
Trehalose.
Datasheet
Please contact us to get it.
Expression Region
2-144
Protein Length
Full Length of Mature Protein
Species
Homo sapiens (Human)
Target Names
Target Protein Sequence
AASKVKQDMPPPGGYGPIDYKRNLPRRGLSGYSMLAIGIGTLIYGHWSIMKWNRERRRLQ
IEDFEARIALLPLLQAETDRRTLQMLRENLEEEAIIMKDVPDWKVGESVFHTTRWVPPLI
GELYGLRTTEEALHASHGFMWYT
Uniprot No.
Target Background
Function
NDUFA13 is an accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), not believed to be directly involved in catalysis. Complex I facilitates electron transfer from NADH to the respiratory chain, with ubiquinone as the likely immediate electron acceptor. NDUFA13 is implicated in interferon/all-trans-retinoic acid (IFN/RA)-induced cell death, an apoptotic activity inhibited by interaction with viral IRF1. It also prevents the transactivation of STAT3 target genes and may play a role in CARD15-mediated innate mucosal responses, regulating intestinal epithelial cell responses to microbes.
Gene References Into Functions
- Recombinant adenovirus expressing GRIM-19 (rAd-Grim-19) may regulate tumor cell growth and apoptosis, improving survival in esophageal tumor-bearing mice. [PMID: 29488605]
- Low GRIM19 expression is associated with radiation resistance in osteosarcoma. [PMID: 30005830]
- GRIM-19 may predict prognosis and disease recurrence in high-grade serous carcinoma. [PMID: 29254797]
- Promoting GRIM19 expression may benefit rheumatoid arthritis treatment. [PMID: 29306209]
- MiR-6743-5p may function as an oncomiR in glioma by targeting GRIM-19 and STAT3. [PMID: 29074558]
- GRIM-19 suppresses proliferation and invasion of cutaneous squamous cell carcinoma cells by downregulating STAT3 signaling. [PMID: 28926927]
- Mitochondrial GRIM-19 is a potential prognostic biomarker and therapeutic target for STAT3-dependent gastric cancer carcinogenesis. [PMID: 27167343]
- GRIM-19 inhibits telomerase activity in cervical cancer cells by inhibiting E6-mediated hTERT promoter transactivation, promoting senescence. [PMID: 27142689]
- NDUFA13 deficiency may be linked to asthenozoospermia through impaired spermatozoa mitochondrial membrane potential, increased apoptosis, and reactive oxygen species. [PMID: 27789183]
- GRIM-19 overexpression suppresses hepatocellular carcinoma (HCC) growth and downregulates AKT1 expression, suggesting a role in negatively regulating the PI3K/AKT pathway. [PMID: 25550785]
- Low GRIM-19 expression is associated with paclitaxel resistance in cervical cancer. [PMID: 26810068]
- Metformin-induced AMPKα activation reverses suppressed GRIM-19 expression in H9C2 cells, with a less pronounced effect in HeLa cells. [PMID: 27101310]
- Ki67 and GRIM19 expression correlate with malignancy in thyroid Hurthle cell tumors, potentially differentiating carcinoma from adenoma. [PMID: 26188382]
- Combined GRIM19 and LKB1 expression effectively suppresses breast cancer growth in vitro and in vivo. [PMID: 26458553]
- Aberrant endometrial GRIM-19 expression is associated with adenomyosis through regulation of apoptosis and angiogenesis. [PMID: 26769301]
- A novel NDUFA13 mutation causes mitochondrial Complex I instability and a slowly progressing neurological phenotype. [PMID: 25901006]
- GRIM-19 overexpression suppresses secretion of urokinase-type plasminogen activator (u-PA), matrix metalloproteinase (MMP)-2, MMP-9, and vascular endothelial growth factor (VEGF). [PMID: 25955394]
- GRIM-19 expression is strongly associated with colorectal cancer progression and may serve as a prognostic biomarker. [PMID: 26363526]
- GRIM-19 overexpression in oral squamous cell carcinoma cells inhibits cell proliferation, migration, and invasion in vitro and tumor growth in vivo. [PMID: 25174621]
- GRIM-19 promoter hypermethylation may contribute to head and neck carcinogenesis by promoting cell proliferation and regulating metabolic activity. [PMID: 25575809]
- Combined ADAM10 siRNA and GRIM19 expression significantly inhibits HepG2 cancer cell proliferation, migration, and invasion. [PMID: 25242535]
- GRIM-19 deficiency in villi may be associated with miscarriage through increased apoptosis and impaired angiogenesis. [PMID: 25455534]
- GRIM-19 may regulate normal cervical tissue differentiation, with decreased expression potentially resulting from HR-HPV infection and subsequent malignant transformation. [PMID: 24690422]
- GRIM-19 mutations are associated with oral squamous cell carcinoma. [PMID: 23851499]
- GRIM-19 is involved in H5N1 virus-induced macrophage apoptosis. [PMID: 23529854]
- Silencing survivin and overexpressing GRIM-19 inhibits Hep-2 laryngeal cancer cell growth and induces apoptosis in vitro and in vivo. [PMID: 24133585]
- GRIM-19 downregulation promotes HIF1α synthesis. [PMID: 23580587]
- GRIM-19, NDUFS3, and extracellular matrix element expression correlate with the invasive capabilities of breast cancer cell lines. [PMID: 23630608]
- GRIM-19 downregulation is associated with STAT3 overexpression in breast carcinomas. [PMID: 23618357]
- GRIM-19 expression correlates with histological grading and p-STAT3 in hepatocellular carcinoma. [PMID: 22492280]
- GRIM-19 expression in lung cancer is related to histological type and clinical stage. [PMID: 22573109]
- Tumor-derived GRIM-19 mutations disrupt its anti-STAT3 activity and promote oncogenesis. [PMID: 23386605]
- STAT3 import into mitochondria depends on GRIM-19, a component of the electron transport chain. [PMID: 23271731]
- GRIM-19 inhibits the STAT3 signaling pathway and sensitizes gastric cancer cells to radiation. [PMID: 23124042]
- GRIM-19 plays a critical role in the initiation and invasive progression of hepatocellular carcinoma. [PMID: 22105514]
- GRIM-19 expression is lower in gliomas and negatively correlates with malignancy; downregulation enhances cell proliferation and migration, while overexpression has the opposite effect, acting through a non-STAT3 pathway. [PMID: 21827581]
- GRIM-19's function in cancer development. [PMID: 21664299]
- Survivin and GRIM-19 expression correlate with prostate cancer pathogenesis. [PMID: 21351527]
- GRIM-19 blocks the E6/E6AP complex and synergistically suppresses cervical tumor growth with p53. [PMID: 21765936]
- Reduced GRIM-19 mRNA and protein levels are observed in lung adenocarcinoma tissues. [PMID: 21040996]
- GRIM-19 expression is downregulated in non-small cell lung cancer. [PMID: 19622307]
- The N-terminus of GRIM-19 is crucial for its tumor-suppressive actions. [PMID: 20595633]
- Alternatively spliced GRIM-19 mRNA is associated with kidney cancer. [PMID: 20505682]
- GRIM-19/CDKN2A synergistically suppresses cell cycle progression by inhibiting E2F1-driven gene expression. [PMID: 20522552]
- GRIM-19 loss is associated with invasion and metastasis of human gastric cancer. [PMID: 20478305]
- Restoring GRIM-19 levels re-establishes control over STAT3-dependent gene expression and tumor growth in vivo. [PMID: 19642906]
- Viral interferon regulatory factor 1 modulates interferon/retinoic acid-induced cell death through interaction with GRIM19. [PMID: 12163600]
- GRIM-19 inhibits signal transducer and activator of transcription 3 (STAT3). [PMID: 12867595]
- GRIM-19 is a key component in NOD2-mediated innate mucosal responses, regulating intestinal epithelial cell responses to microbes. [PMID: 15753091]
- IFN-β- and tretinoin-induced GRIM-19 upregulation occurs during focal cerebral ischemia. [PMID: 17523870]
Database Links
Involvement In Disease
Hurthle cell thyroid carcinoma (HCTC)
Protein Families
Complex I NDUFA13 subunit family
Subcellular Location
Mitochondrion inner membrane; Single-pass membrane protein; Matrix side. Nucleus.
Tissue Specificity
Widely expressed, with highest expression in heart, skeletal muscle, liver, kidney and placenta. In intestinal mucosa, down-regulated in areas involved in Crohn disease and ulcerative colitis.
Quick Inquiry
Personal Email Detected
Please use an institutional or corporate email address for inquiries. Personal email accounts ( such as Gmail, Yahoo, and Outlook) are not accepted. *
