RIMKLA

Last update 08 May 2025

Basic Info

Synonyms

FAM80A, MGC47816, N-acetylaspartylglutamate synthase A

+ [8]

Introduction

Catalyzes the synthesis of N-acetyl-L-aspartyl-L-glutamate (NAAG) and N-acetyl-L-aspartyl-L-glutamyl-L-glutamate.

100 Clinical Results associated with RIMKLA

100 Translational Medicine associated with RIMKLA

0 Patents (Medical) associated with RIMKLA

Literatures (Medical) associated with RIMKLA

01 Apr 2025·Magnetic Resonance in Medicine

Proton‐free induction decay MRSI at 7 T in the human brain using an egg‐shaped modified rosette K‐space trajectory

Article

Author: Andronesi, Ovidiu C. ; Hingerl, Lukas ; Strasser, Bernhard ; Hangel, Gilbert ; Marjańska, Małgorzata ; Klauser, Antoine ; Blömer, Simon ; Motyka, Stanislav ; Bogner, Wolfgang

AbstractPurposeProton (1H)‐MRSI via spatial‐spectral encoding poses high demands on gradient hardware at ultra‐high fields and high‐resolutions. Rosette trajectories help alleviate these problems, but at reduced SNR‐efficiency because of their k‐space densities not matching any desired k‐space filter. We propose modified rosette trajectories, which more closely match a Hamming filter, and thereby improve SNR performance while still staying within gradient hardware limitations and without prolonging scan time.MethodsAnalytical and synthetic simulations were validated with phantom and in vivo measurements at 7 T. The rosette and modified rosette trajectories were measured in five healthy volunteers in 6 min in a 2D slice in the brain. An elliptical phase‐encoding sequence was measured in one volunteer in 22 min, and a 3D sequence was measured in one volunteer within 19 min. The SNR per‐unit‐time, linewidth, Cramer‐Rao lower bounds (CRLBs), lipid contamination, and data quality of the proposed modified rosette trajectory were compared to the rosette trajectory.ResultsUsing the modified rosette trajectories, an improved k‐space weighting function was achieved resulting in an SNR per‐unit‐time increase of up to 12% compared to rosette's and 23% compared to elliptical phase‐encoding, dependent on the two additional trajectory parameters. Similar results were achieved for the theoretical SNR calculation based on k‐space densities, as well as when using the pseudo‐replica method for simulated, in vivo, and phantom data. The CRLBs of γ‐aminobutyric acid and N‐acetylaspartylglutamate improved non‐significantly for the modified rosette trajectory, whereas the linewidths and lipid contamination remained similar.ConclusionBy optimizing the shape of the rosette trajectory, the modified rosette trajectories achieved higher SNR per‐unit‐time and enhanced data quality at the same scan time.

01 Mar 2024·Journal of Inherited Metabolic Disease

NAAG synthetase deficiency has only low influence on pathogenesis in a Canavan disease mouse model

Article

Author: Becker, Ivonne ; Wang-Eckhardt, Lihua ; Eckhardt, Matthias

AbstractCanavan disease (CD) is a leukodystrophy caused by mutations in the N‐acetylaspartate (NAA) hydrolase aspartoacylase (ASPA). Inability to degrade NAA and its accumulation in the brain results in spongiform myelin degeneration. NAA is mainly synthesized by neurons, where it is also a precursor of the neuropeptide N‐acetylaspartylglutamate (NAAG). Hydrolysis of this peptide by glutamate carboxypeptidases is an additional source of extracellular NAA besides the instant neuronal release of NAA. This study examines to what extent NAA released from NAAG contributes to NAA accumulation and pathogenesis in the brain of Aspanur7/nur7 mutant mice, an established model of CD. Towards this aim, Aspanur7/nur7 mice with additional deficiencies in NAAG synthetase genes Rimklb and/or Rimkla were generated. Loss of myelin in Aspanur7/nur7 mice was not significantly affected by Rimkla and Rimklb deficiency and there was also no obvious change in the extent of brain vacuolation. Astrogliosis was slightly reduced in the forebrain of Rimkla and Rimklb double deficient Aspanur7/nur7 mice. However, only minor improvements at the behavioral level were found. The brain NAA accumulation in CD mice was, however, not significantly reduced in the absence of NAAG synthesis. In summary, there was only a weak tendency towards reduced pathogenic symptoms in Aspanur7/nur7 mice deficient in NAAG synthesis. Therefore, we conclude that NAAG metabolism has little influence on NAA accumulation in Aspanur7/nur7 mice and development of pathological symptoms in CD.

01 Oct 2023·iScience

mNSCs overexpressing Rimkla transplantation facilitates cognitive recovery in a mouse model of traumatic brain injury

Article

Author: Pang, Ying ; Zhang, Chunyu ; Zhang, Jing ; Chen, Xu ; Wang, Rui ; Cheng, Meng ; Ji, Tongjie ; Zhong, Chunlong ; Liu, Min

N-acetyl aspartyl-glutamate (NAAG) is easily inactivated for the hydrolysis of NAAG peptidase on the surface of glial cells, thereby losing its endogenous neuroprotective effect after traumatic brain injury. In this study, lentiviral vectors were used to over express/knock out NAAG synthetase II (Rimkla) in mouse embryonic neural stem cells (mNSCs) in vitro and these mNSCs were transplanted at the lesion site in a mouse model of controlled cortical impact (CCI). In vivo experiments showed that transplantation of mNSCs overexpressing Rimkla regulated glutamate-glutamine cycling between adjacent astrocytes and neurons in the subacute phase of CCI, thereby enhancing support for neuronal metabolism and promoting neuronal synaptic repair in the hippocampal CA3 region. Taken together, these findings demonstrate that transplantation of neural stem cells overexpressing Rimkla can effectively increase the NAAG concentration in local brain regions, which opens up new ideas for the maintenance of NAAG neuroprotective effects after TBI.

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